|Year : 2015 | Volume
| Issue : 6 | Page : 41-88
Selected Abstracts of IANCON 2015 (Poster)
|Date of Web Publication||6-Oct-2015|
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
. Selected Abstracts of IANCON 2015 (Poster). Ann Indian Acad Neurol 2015;18, Suppl S2:41-88
Poster Session 01, Friday, October 02, 2015,
8: 30 AM - 10:30 AM
Dr. Vimla Vermani Hall (Hall D): Clinical Neurophysiology
Snake Bite with Toxic Demyelination
Manivannan, Justin, Chezhian, Ganesan, Amalraj Iyadurai, Senthur Rajapandian, Sritharan
Madurai Medical College, Madurai, Tamil Nadu, India
Introduction: Snakebite is an important cause of mortality and morbidity in India. Acute disseminated encephalomyelitis is also reported following treatment with anti snake venom. Case Vignette: A 45 years old female patient presented with history of snakebite on her left hand. She was treated outside for 5 days with Anti snake venom FFP and referred. On admission she had history of bleeding gums decreased urine outputhemoptysis and hematuria of one day duration. On day - 6 she developed weakness of both lower limbs and on day- 7 she had upper limb weakness and respiratory distress. On examination the patient was conscious. Her vitals were stable. Higher mental function cranial nerves normal. Motor system handgrip-weak bilaterally powerboth LL 0/5. Plantar no response. DTR- triceps jerk knee jerk ankle jerk were absent on both sides all other DTR were normal. Sensory system all modalities decreased below D4. Autonomic dysfunction was present. There was no cerebellar signno meningeal sign. The spinal vibration was decreased below T2. Investigations: RBS- 234mgs%. Urea-67. Creatinine-1.4 mgs%. Total bilirubin-2.4mgs. Indirect bilirubin -1.2 mgs%. Clotting timeΛƒ20 minutes. Hb-7.4mgs%. Platelet - 21000/cu mm. Prothrombin time 21.6 seconds. MRI Cervical spine showed T2 hyperintensity from C5 to D5 level and reported as possibility of toxic demyelination. MRI Brain &MRAMRV-Normal. Patient was treated with IV Methyl prednisolone blood and FFP transfusion and other supportives. Mechanical ventilation and peritoneal dialysis were done. Conclusion: Our patient had coagulopathy and renal failure following hemotoxic snake bite. At the end of first week she developed weakness of all 4 limbs with respiratory distress. MRI showed T2 hyperintense signals in cervical and dorsal spinal cords. ADEM can occur following treatment with Anti snake venum. But our case had direct toxic demyelination of spinal cord after snake bite. This case is presented for its rarity.
Variant of Miller Fisher Syndrome
Chezhian, Jason Ambrose, Senthur Rajapandian, Birla Pavalam, Muthukumar, Justin, Sritharan, Manivannan
Madurai Medical College, Madurai, Tamil Nadu, India
Introduction: Miller fisher syndrome is a rare variant of Guillain-Barre syndrome characterized by the acute development of ataxia ophthalmoparesis and areflexia. Different variants of Miller Fisher syndrome arepresent. Case Vignette: A 30 years old male patient presented with difficulty in getting up from sitting posture and difficulty in raising arms above shoulder of 2 days duration. He had tingling sensation over both palms and feet. He also had unsteadiness while walking and inability to close both eyes with deviation of angle of mouth towards right side. The above complaints are of acute onset and progressing in nature. On Examination the patient was conscious. His vitals were stable. Higher function and speech were normal. He had external ophthlmoplegia; pupils equal and reacting. There was bilateral LMN facial nerve palsy. Flail quadriparesis with power of 4/5 in all 4 limbs was present without neck muscle weakness. Generalized areflexia diminished vibration sense in legs positive Romberg's incordination of limbs were the other clinical features. Routine blood tests including complete hemogram renal and liver function testselectrolytes thyroid profile CPK were normal. HIV and ANA negative. NCS showed normal CMAPF wave latency; SNAP not obtained in both upperlimbs and lowerlimbs. CSF analysis showed albuminocytological dissociation. MRI brain with whole spine screening was normal.. Patient was treated with IV Methylprednisolone and other supportives. Conclusion: Our patient presented with all the classical features of Miller Fisher syndrome namely opthalmoplegiaataxia and areflexia. In addition he had quadriparesis and facial weakness. Our case is a variant of Miller Fisher Syndrome with GBS overlap. The case is presented for its rarity.
Recurrent Amsan Variant of Guillain Barre Syndrome
Thahira T P, Chaithra S P, J B Agadi, Archana N B, Karthik N, Praveen Kumar
Bangalore Medical College, Karnataka, India
A 63 year old man presented with a history of acute onset quadriparesis with respiratoryparalysis. He was normokalemic and his Nerve conduction studies revealed absent CMAP s and SNAP s in all sampled nerves. A diagnosis of AMSAN variant of Guillain Barre syndrome was made and he was given a course of Ivig. Howeverhe did not improve in motor power and continued to remain on ventilator for a further period of six months. On going through the previous medical records we found that he had a similar illness 3 years back for which he had received Ivig. Nerve conduction study reports then also revealed AMSAN variant of Guillain Barre Syndrome. The patient made a prolonged recovery over a period of 8 months. Review of literature showed that GB syndrome may recur after a variable interval in 1-6% of patients. However we could not find any other published cases of recurrent AMSAN variant of G B syndrome. We presume that this is the first case report of recurrent AMSAN from India.
A Short Series of Chronic Immune Sensory Polyradiculopathies (CISP) With Long Term FollowUp
Bhagyadhan. A. Patel, SV Khadilkar, Nilesh Nadkarni
J.J. Hospital and Grant Govt. Medical College, Mumbai, India
Very rarely immune mediated demyelinating radiculopathies remain restricted to only the proximal roots. Less than a dozen such cases have been reported in world literature till date. Such unusually localised forms of immune mediated demyelinating radiculopathies (CISP)s can surprise and perplex clinicians. We present here case series of 3 patients who presented with very slowly progressive proximal weakness and sensory ataxia. Examination showed generalised areflexia with large fibre sensory loss in lower limbs. However all CMAPs and SNAPs parameters in all three of them were within normal range. But their F-waves and H- reflex were attenuated. MRI neurography with contrast showed diffuse contrast enhancement of roots and CSF examination showed gross albumin-cytological dissociation. Chronic inflammatory sensory polyradiculopathy is now a distinct and localised form of immune mediated neuropathies which is frequently underdiagnosed and potentially treatable. Long term treatment results are available in two patients showing some degree of benefit.
Two cases of Non-diabetic Radiculoplexus Neuropathy
Surendramohan T, Thaseen A, Ramakrishnan V, Chandramouleeswaran V, Bhanu K
Institute of NeurologyMadras Medical College, Kanchipuram, Tamil Nadu, India
Introduction: Our cases presented with sub-acute onset of weakness and variable sensory loss of both lower limbs. Case 1: One month back a 64 year old man developed weakness of left footthen severe pain and weakness of left hipthigh and legfollowed by numbness of left thigh and leg. Similar symptoms occured in right lower limb 1 year back. There was hypotoniadepressed jerks in both lower limbsweakness of hipknee and anklesdecreased pain left thighboth legs and feet. Lower limb CMAPs not obtainedEMG -neurogenic pattern. CSF protein-78mg%acellularCSF-CMV-negative. RA factor ANAANCA-negativeMRI-normal. Biopsy -sural-chronic axonal/demyelinating neuropathy muscle-neurogenic atrophy. He was treated with weekly pulses of methyl prednisolone. Case 2: Three years back60 year old man developed numbness over left hipbuttock and outer thigh and over the right hip 1 month later. Weakness of both hipsknees and ankles began and progressed over past 3 years with LBA. Lower limb was hypotonic with absent jerkspower hip 3knee 2 and ankle 3with decreased sensations below D10. MRI brain/spine TFTserum B12 were normal. NCS-reduced lower limb CMAP amplitudes with normal latencies and NCV inconsistent or absent F waves absent SNAPs. CSF acellular with protein 51mg%. He was treated with oral steroids. Discussion: Lumbosacral radiculoplexus neuropathy (LRPN) is an uncommon condition featuring asymmetrical lower extremity pain weakness and muscle atrophy affecting thigh commonly with mild sensory symptoms. Originally described in diabeticsrecently it is recognized in non-diabetics. Conclusion: The clinical diagnosis of LRPN should also be entertained in non-diabetic subjectsthough it occurs less frequently follows a similar clinical course
Assessment Of Sleep Spindle Density Among Genetically Positive Spinocerebellar Ataxias Type 1 2 And 3 Patients: A Comparative Study
Ragasudha Botta, Seshagiri Doniparthi, Arun Sasidharan, Ravi Yadav, Pramod Pal, Sanjeev Jain, Bindu M. Kutty
National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
Introduction: Spinocerebellar ataxias (SCA) are hereditary progressive ataxias with degeneration of brain stem and cerebellum. Sleep problems are observed in SCA patients. Degeneration of thalamus is present in SCA2 and SCA3. Sleep spindles are distinctive EEG oscillations(11-16Hz) generated by thalamo-cortical interplay during NREM stage. Objectives: To compare sleep spindle densities (SSDs) of genetically proven SCA1SCA2 and SCA3 patients. Methodology: Prospectively genetically confirmed cases of SCA and controls were recruited from Neurology outpatient and inpatient services at NIMHANS Bangalore. Ethical clearance was obtained. Sleep quality was assessed using various rating scales. Overnight polysomnography was performed at Sleep lab in department of Neurophysiology. SSDs were analyzed using neuroloop gain plugin of Polyman v1.15 software and averaged across the NREM sleep using custom scripts written in MATLAB-2013a. Results: : Eighteen SCA12 & 3 patients(6 in each group) of both gender between ages 18-47 years and six age/gender matched controls were included. Mean age of SCA1 patients was 39.175.42SCA2 patients was 30.809.45and SCA3 patients was 35.436.35 years. This study shows significant SSD differences in SCA 2 & 3 patients when compared to controls. NREM SSD values(percentage Neuroloop gain) are 16.91% in SCA10% in SCA21.2% in SCA3 and 59.49% in controls. No significant differences observed in SSDs between SCA1,SCA2 and SCA3 patients. Conclusion: This study shows "thalamic switch" disruption observed as reduced SSDs in SCA2 & SCA3. Sleep spindle deficits could act as one of the biomarkers of ongoing neurodegeneration in the thalamic circuitry of SCA patients. This novel finding needs to be confirmed in larger cohort.
Phrenic nerve conduction study in the early stage of Guillain Barre syndrome as a predictor of respiratory failure
Barun Kumar Sen, Asit Kumar Senapati, Alak Pandit
Bangur Institution of Neurosciences IPGME&R, Kolkata, West Bengal, India
Introduction: In Guillain-Barre΄s syndrome (GBS) up to 30% of patients require assisted ventilation due to neuromuscular respiratory failure; its main cause is weakness of the diaphragm. Successful management mandates anticipation prompt recognition and optimal treatment of neuromuscular respiratory failure in GBS. Aims: To evaluate the frequency of phrenic nerve abnormalities in the early Guillain-Barre syndrome and phrenic nerve conduction study as a predictor of respiratory failure in the early stage of Guillain Barrae syndrome. Material and Methods: Forty six patients of GBS (above 18 yrs) admitted within 14 days of onset to BIN IPGMER Kolkata from Jan 2014 to May 2015 were studied. The clinical parameters (median age gender antecedent infection time to peak disability bifacial weakness upper limb weakness bulbar paralysis neck weakness) and electrodiagnostic studies like motor nerve conduction phrenic nerve conduction study (CMAP amplitude distant latency duration) were done. Results: The total 46 cases of GB syndrome clinical parameters like median age gender antecedent infection bifacial weakness upper limb weakness did not influence the development of neuromuscular respiratory weakness but time to peak disability bulbar paralysis neck weakness influenced. Phrenic nerve CMAP latencies amplitude duration were significantly different between GBS patients with respiratory failure and without respiratory failure. Six patients with the sum of phrenic nerve latency longer than 18 ms and the sum of bilateral CMAP amplitude smaller than 0.2 mV and longer CMAP duration required respiratory assistance. Conclusion: Phrenic nerve conduction study was found to be a sensitive parameter in assessing the severity of the disease and predicting impending respiratory failure in GB syndrome patient.
MUNE - A Diagnostic Tool in the Early Diagnosis of Carpal Tunnel Syndrome
Nilesh Chaudhary, Shyam Jaiswal, Muralidhar Reddy, Murthy JMK, E S S Karan, N Ravi
Care Hospital, Hyderabad, India
Background: The diagnosis of carpal tunnel syndrome (CTS) is often based on the electro diagnostic data. The sensitivity and specificity of conventional electrodiagnostic studies is 80.2 (71.3-89.0) and 78.7 (66.4-91.1) respectively. Motor unit number estimation (MUNE) is a new technique and not well studied in CTS. Methods: Study subjects included 37 patients with 68 symptomatic hands who fullfilled the clinical diagnostic criteria of CTS proposed by American Academy of Neurology (AAN) and 32 healthy controls. In addition to conventioal electrodiagnostic (AAN criteria) tests, Combined Sensory Index (CSI) and MUNE in abductor pollices bravies (APB) were also studied. Statstical analysis included conventional stests. ANOVA and post hoc correlation coefficient, and Receiver Operating Characteristic Curve (ROC curves). Results: Of the 68 symptomatic hands, 64 (94%) hands fullfilled the AAN electrodiagnostic criteria for CTS. Whereas decreased number of motor units (MUs) on MUNE in APB observed was in 66 (96%) hands. All the 5 hands with normal palmar-median sensory latency difference had abnormal CSI. Of the 46 (71%) hands with normal compound action potential (CMAP), 20 (43%) had decreased number of MUs on MUNE in APB. There was linear relation between motor latency and MUNE abnormalities. The sensitivity and spcificity for various parameters based on ROC data were: 114.5 for MUNE (sensitivity 98.5%, specificity 96.7%); 10.95 mV for CMAP amplitude (sensitivity 73.1%, specificity 65.6%); for difference in palmar median and ulnar sensory latency (sensitivity 90%, specificity 88 %) and for CSI (sensitivity 92%, specificity 94 %). Conclusion: In this study MUNE and CSI were found to have high diagnostic sensitivity and specificity for CTS when compared to conventional electrodiagnostic tests.
Significance of Bifid Median Nerve In Patients With Carpal Tunnel Syndrome
Dileep R, Benjamin Ramacha, Chitra. P, Thomas Iype
Government Medical College, Thiruvananthapuram, Kerala, India
Aim: To determine frequency of bifid median in CTS patients its ultrasound characteristics& response to therapy. Methodology: Sixty patients (102 hands) with symptoms of CTS (electrophysiologically confirmed) were included. 30 healthy volunteers (60hands) were taken as control. All patients & controls underwent wrist ultrasound to identify bifid median in carpal tunnel and accompanying abnormalities like persistent median artery. Cross sectional area of median nerve was measured at carpel tunnel (CSA1). Patients who were willing for surgery underwent release surgery & rest was given local steroid injection. Patients were assessed at baseline & 3 months using Boston carpal tunnel questionnaire (BCTQ) visual analogue scale(VAS) electrophysiology and ultrasound. Results: We found more bifidity among CTS patients (11 patients 18.33%) than controls (2 controls 6.67%)(p=0.000Ο‡2=13). Bilateral CTS was seen in 5 patients (45%)with bifid median compared to 43(87.7%) among nonbifid patients. In patients with bifid median nerve non dominant hand was more severely involved whereas in non-bifid patients dominant hand was more involved. Majority of patients in bifid(75%)&nonbifid group(65%)had moderate CTS(electrophysiologically). Ultrasound in bifid median showed a larger radial branch in 64.7% cases &66.7% controls. Persistent median artery was present in 8 hands in cases (50%) and 2 in controls (66.7%). CSA1 of nonbifid CTS patients was (14.4 Β 5.04) mm2 &of bifid CTS patients was (11.8Β 1.96)mm2.. Out of eleven patients6 underwent release surgery& 5 had local steroid injection. There was significant improvement in VASBCTQ parameters motor latency at 3 months in both surgery & steroid arm. Conclusion: This study shows relative high frequency of bifid median nerve in CTS patients. The clue for doubting bifidity as a cause of CTS may be predominant nondominant hand involvement. Symptomatic benefit at 3 month after surgery &steroid injection was similar.
HRUS: Features of Sciatic Neuropathy in Hansens Disease
Syed Osman, Muralidhar Reddy Y, Nilesh Vijay Chaudhary, Shyam K Jaiswal, Murthy JMK
The Institute of Neurological Sciences Care Hospital, Hyderabad, India
Introduction: High-resolution sonography (HRUS) has been shown to be a useful noninvasive tool in the diagnosis of leprosy neuropathy. Epineurial thickening and Fascicular distortion are good biomarker of the extent of nerve damage in leprosy. This report describes HRUS findings in a leprosy patient with sciatic neuropathy. Case: A 42-yr-old male presented with progressive left foot drop of 2 months duration. No history of sensory symptoms and symptoms referable to other parts of neuroaxis. Neurological examination revealed bilaterally enlarged greater auricular nerves hypo-aesthetic patch over anterior aspect of left leg and left foot drop. Electrodiagnostic features were consistent with left sciatic neuropathy. HRUS of left sciatic nerve at the level of proximal and mid thigh revealed enlarged left sciatic nerve with partly distorted fascicular architecture in common peroneal nerve (CPN) division and retained architecture in tibial division. HRUS of left sciatic nerve at distal thigh and knee revealed completely effaced fascicular architecture of left CPN division and normal tibial division. HRUS of both left and right greater auricular nerve revealed enlarged greater auricular nerves in transverse and longitudinal section with thickened epineurium and effaced fascicular architecture. HRUS of right sciatic nerve left and right ulnar nerve showed normal fascicular architecture. Conclusion: HRUS is a useful tool to diagnose nerve involvement in leprosy and also to assess the treatment response. The unusual features in this patient are selective involvement of CPN division of sciatic nerve and absence of involvement of ulnar nerves.
Poster Session 02, Friday, October 02, 2015,
8: 30 AM - 10:30 AM
Dr. Vimla Vermani Hall (Hall D): Autoimmune Disorders
Posterior reversible encephalopathy syndrome (PRES) associated with i.v Methyl prednisolone and Methylcobalamine
Ahmad Maqsood Dar, Mukesh Kumar, Abdul Hamid Malik, Vasundhara Saikia, Khusbu Goel, Joy Dev Mukherji
Background: posterior reversible encephalopathy syndrome (PRES) is a novel clinical entity commonly presenting with headache seizures and visual changes. PRES syndrome associated with I.V methyl prednisolone and Vit B12 is a rare entity. 51 years lady presented in casualy with severe anemia and paraparesis. Examination revealed severe anemia and paraplegia. Biochemically she had sever vitamin B12 and iron deficiency. MRI spine showed long segment myelitis in dorsal cord. She received intravenous iron vitamin B12 and methylprednisolone pulse therapy along with supportive medications. After 5 days she developed behavioral changes followed by seizure. MRI brain showed bilateral diffuse white matter edema with faint restricted diffusion in cortical and subcortical region with possibility of posterior reversible encephalopathy syndrome (PRES). She further received pulse methylprednisolone and continued on dexamethasone. She recovered significantly. She developed acute deep venous thrombosis and started on anticoagulation. In her last outpatient visit she started standing with support and coherent and alert in all activities. This case is unique as she developed PRES on vitamin B12 and methylprednisolone. Near complete clinical recovery in a month on tapering steroid confirms possibility of PRES. Review of literature The first patient to develop PRES syndrome associated with methyl prednisolone pulse therapy was a six years old child with juvenile idiopathic arthritis1. The second case case of PRES syndrome associated with methyl prednisolone pulse therapy was reported by Faruk incecik M. Ozlem Herguner et al. 2 in a 8 year boy who presented with fever and pallor for 2 days and was diagnosed as Idiopathic Auto immune Hemolytic Anaemia. The patient started i.v methyl prednisolone at a dose of 30 mg /kg body weight was given started. On day 4 the patient suddenly developed headache confusion and seizures and was diagnosed as PRES Syndrome. Conclusion: Take home message of this presentation is that the possibility of PRES syndrome while using i.v methyl prednisolone and Vit B12 should be kept in mind as any of the two could be a contributing factor for PRES syndrome.
Tubercular Long Extensive Transverse Myelitis (LETM)
Suman Kushwaha, Lomesh Bhirud, Siddharth Maheswari
Institute of Human Behavior and Allied Sciences, Delhi, India
Objective: Tubercular LETM is under diagnosed cause of LETM. Background: Tuberculous meningitis is the most common cause of meningitis in adults in tropical countries. Mycobacterium Tuberculosis as an etiological cause of long extensive transverse myelitis is an uncommon entity in evaluation of patients of LETM however should always be kept possibility as treatable condition. Methods: Clinical History Neurological Examination investigations including CSF study and Neuroimaging. Follow up of the patients after starting anti-tuberculous therapy (ATT) for next one year. Results: Among 10 patients presented with LETM in year 2012-2014 in Neurology department of IHBAS Delhi; 6 were female and 4 were male. 9 patients presented with paraparesis and 1 with quadriparesis. All patients were undergone routine haematological renal liver functions tests and CSF studies including TB PCR and M. TB culture. All patients were screened for autoimmune illness with ANA anti-dsDNA antibody including anti-aquaporin 4 antibody. They also evaluated with MRI Brain imaging and electrophysiological testings including Visual evoked potentials (VEP) and Brainstem auditory evoked response (BAER) studies. Following thorough evaluation patients those with definite tuberculos LETM and LETM without definite tuberculous evidence were treated with ATT for next one year. Conclusions: Tuberculosis of spinal cord presenting as LETM should be considered in tropical countries as one of the treatable condition.
Osmotic Demyelination Syndrome and Rhabdomyolysis Secondary To Postpartum Hypernatremia
Deepak N, Archana N B, Jb Agadi, Karthik N, Praveen Kumar
Department of Neurology Bangalore Medical College, Bangalore, India
BMC&RIBengaluruExtra pontine myelinolysis associated with metabolic abnormalities especially hypernatremia is extremely rare disorder. Osmotic demyelination syndrome resulting from postpartum hypernatremia is recently described entity (2010) where young women present with hypernatremic encephalopathyquadriparesis and rhabdomyolysis with myelinolysis on imaging. A 20 year-old lady presented with fever and disorientation of 5 days and generalized tonic clonic seizures of 1-day duration. She had undergone a lower segment caesarean section3 weeks prior to this and had an uneventful postoperative periodand was keeping well for next 1 week. On examination she was febrile vitals were stable but was drowsy flexing limbs and opening eyes to pain with no verbal response. Limbs were flaccidareflexic and plantars were unresponsive. Biochemical evaluation revealed a sodium level of 182 meq/l serum CPK -2207 U/L urea-135 mg/dl and creat-4.3 mg/dl. MRI brain showed T2/FLAIR hyperintensities in bilateral basal ganglia external capsule middle cerebellar pedunclesposterior internal capsuleand subcortical white matter suggestive of extrapontinemyelinoysis. Patient underwent correction of metabolic abnormalities however progressively worsened in sensorium and motor weakness and succumbed to her illness over next 7 days. Clinicians must be aware of this entity of postpartum hypernatremia due to water restriction and diabetes insipidus which should be looked for and treated at the earliest as mortality remains high (>50%).
Hypertrophic spinal pachymeningitis: Rare manifestation of a rare disease- case report
Subramanian R,Abid M, Shanmuga Sundaram N, Balasubramanian S, Bhanu K
Institute of NeurologyMMC, Chennai, India
Introduction: Hypertrophic pachymeningitis is a rare chronic fibrosing inflammatory disease characterized by marked diffuse thickening of dura matter either cranial or spinal that causes progressive neurologic deficit. Isolated spinal form is uncommon. Here we report a case of hypertrophic spinal pachymeningitis with possible IgG4 related disease. Case vignette: 41 years old female presented with recurrent episodes of dorsal myelopathy above D4 level clinically with near-normal recovery in between the episodes with treatment. Investigations revealed elevated ESR normal peripheral smear study. Viral markers serum thyroid profile ACE CK LDH calcium were normal. Serum VDRL was non-reactive. ANA ANCA RF were negative. Serum IgE level was elevated and IgG4 level was normal. CSF showed <5 lymphocytes protein-120 mg% viral studies VDRL and TB-PCR negative. MRI showed inflammatory contrast enhancing anterior epidural mass extending from D1-D12 spinal cord and periaortitis with no significant abnormality in brain. Whole body PET-CT revealed paratracheal lymph nodes and perivasculitis involving whole of aorta both common and internal carotid arteries and bilateral common iliac arteries. Histopathological examination of cord lesion showed diffuse infiltration of lymphocytes and macrophages suggestive of pachymeningitis. We concluded the possibility of IgG4 related disease excluding other conditions causing such complex of organ involvement. Discussion: IgG4 related disease is an immune mediated inflammatory disorder that may involve every organ system with the hallmark of lymphoplasmacytic infiltration with a predominance of IgG4 positive plasma cells. Patients can have normal serum IgG4 levels due to prozone phenomena. Conclusion: We report this case for its rarity and for documentation.
Chronic Inflammatory Demyelinating Polyneuropathy associated with Hepatitis B virus Infection
Vasundhra Saikia, Mukesh Kumar, Abdul Hamid Malik, Ahmed Maqsood Dar, Khusbu Goel
New Delhi, India
Background: Neurological manifestations of Hepatitis B infection are rare. Only few case reports showing association of HBV infection with Demyelinating Neuropathy (AIDPCIDP) have been reported. The pathogenesis is not known but most likely it is immune complex mediated. Objective: To show the association of Chronic Inflammatory Demyelinating polyneuropathy with Hepatitis B infection. Material and Methods: We report a single case of Chronic Inflammatory Demyelinating polyneuropathy with Hepatitis B infection. A 47 years old diabetic man was admitted in hospital with 10 days history of insidious onset rapidly progressive flaccid quadriparesis with bilateral facial and neck flexor weakness and jaundice. On neurological examination he had quadriparesis weak flexion at neck and bilateral lower motor neuron type 7th nerve palsy. Deep tendon reflexes were absent. He had jaundice but no asterixis. Serum Biochemistry revealed hyperbilirubinaemia hypergamaglobulinaemia and elevated liver enzymes. HBsAg and Anti HbcAb were positive with raised HBV DNA levels. Immunofixation was negative for M-spike. Neurophysiological examination demonstrated demyelinating polyneuropathy. His MRI (2)of Brain and whole spine were normal. He was diagnosed with Acute Inflammatory Demyelinating Polyneuropathy and given IVIG along with antiviral. His hepatic dysfunction improved on antiviral and was discharged. However after 7 days of discharge he was readmitted with history of progressive dyspnea and altered sensosium of 2 days duration. ABG showed type II respiratory failure with CO2 narcosis. He was intubated and put on ventilator and was managed in ICU. He was started on systemic steroids and Azathioprine. His neurological symptoms and ventilator requirement improved within 2 weeks.
A Case Report of Anti-LGI Encephalitis
Ganesan, Ramu, Thirumalai Rajan, Amalraj Iyadurai, Muthuveeran
Madurai Medical College, Madurai, Tamil Nadu, India
Anti-leucine-rich glioma inactivated-1 (LGI1) limbic encephalitis (LE) is a rare neurological disorder that has a subacute course of progressive encephalopathy and fasciobrachial dystonic seizures. Case Vignette: 41 year old male patient presented with memory disturbances since feb 2015. The memory disturbance was for recent memory with interference in his working place as well as noted by his family members. This was later followed by daytime sleepiness sleep disturbances in the form of sleep bruxism. Patient started developing recurrent episodes of facial grimacing and abnormal arm-twisting movements. He regained consciousness a few minutes later but was disoriented to the time and place. This episodes started to occur in increasing frequency following which patient was referred to us. On examination patient was conscious recent memory impairment present obeyed commands cranial nerves spinomotor systemsensory and cerebellum were normal. Investigations revealed normal RFTsugarLFT & complete hemogram. Serum electrolytes showed hyponatremia EEG showed slowwave activity indicative of an encephalopathic process. An MRI head showed high signalchanges in the hippocampal areas consistent with the clinical diagnosis of limbic encephalitis. CSF analysis including virological tests returned negative including Mycobacterium tuberculosis PCR. He was started on Methyl prednisolone and immunoglobulin intravenously. Anti-VGKC antibodies was sent and were reported as strongly positive (herein to bereferred to as anti-LGI1antibodies ) Discussion: It can present in a similar way to other encephalitic processessuch as those of an infectiousetiology. Anti-LGI1 LE usually involves the medial temporal area which causes memory dysfunction and seizures. Hyponatremia and memory disturbance in addition to a recent history of FBDS could make us to suspect anti-LGI1 LE. Hyponatremia is commonly detected in patients with anti- LGI1 LE. We present this case for its rarity.
Rituximab in Refractory Myastenia Gravis
Kunal Jadhav, K Ramados, R Balakrishnan, Anish Ninan
P.S.G Hospital, Coimbatore, Tamil Nadu, India
Myasthenia gravis is the paradigm of an antibody-mediated disorder and B cells plays an important role in the pathogenesis. Rituximab is a chimaeric monoclonal antibody against CD20 which depletes B cells. Rituximab is widely used in hematological disorders with reasonable safety profile. It also Rituximab is widely used in hematological disorders with reasonable safety profile. It has also been tried in several immune mediated neurological disorders with varying efficacy. There are case reports showing improvement in myasthenia gravis with rituximab. We report three patients with generalized MG refractory to conventional therapies. In all these three patients treatment with rituximab led to a sustained clinical improvement. Rituximab is an evolving option in the treatment of myasthenia gravis. Considering the cost and safety compared to the other standard therapy currently in use (Intravenous Immunoglobulin & Plasmpheresis) it is promising in economically deprived nation like ours. We conclude that preliminary data on the efficacy and safety of rituximab are encouraging and that further studies in MG seem warranted.
Epidemio-Clinical Spectrum of Demyelinating Disorder of Central Nervous System in a Tertiary Care Centre of Eastern India
Nihar Ranjan Biswal, A.K. Mallick, Geeta Mohanty, K.P. Swain
S.C.B Medical College, Cuttack, Odisha, India
Background: Idiopathic inflammatory demyelinating disorders of the central nervous system (IIDCDs) represent a broad spectrum of disorders including Multiple sclerosis (MS) NMO spectrum disorders (NMOSDs) clinically isolated syndromes (CIS) and acute disseminated encephalomyelitis (ADEM). Understanding the varied clinical profile is important for diagnosis & management. It is also important to identify grey areas in categorization Objective: To study the clinical imaging & laboratory profile of patients of IIDCDs & categorize them into specific groups Method: Retrospective case series of 60 pts. Cases effectively categorized into 4 groups: MS NMOSDs CIS & ADEM and their subgroups. Results: 32% categorized as NMOSDs 28.4% as MS 25.9% as CIS & 13.6% as ADEM. Disease course was monophasic in 64.2% & multiphasic in 29.6%. Polysymptomatic clinical presentation was most common followed by pyramidal & optic neuropathy. 23.46% had Optico-spinal presentation; LETM seen in 44% cases. Aquaporin-4 positivity seen in 40% NMOSDs. Discussion: Higher relapse rate & disability in NMOSDs but longer duration of disease & frequent relapses in MS group. 30% CIS fulfilling criteria for MS-DIS. Higher gray matter involvement in MS & ADEM patients. Low Aquaporin-4 seropositivity (2/5) in tested IIDCDs. No significant difference between seropositive & negative pts. In terms of relapses cord length & disability. Conclusion: IIDCDs a spectrum of disorders because of atypical cases optico-spinal involvement in all groups. Clinical features course & severity play a dominant role in categorization.
An unusual presentation of Idiopathic Orbital Inflammatory Syndrome
Meenakumari S, Shaji C V, Kabeer K A
TDMC Alappuzha, Alappuzha, Kerala, India
Idiopathic Orbital Inflammatory Syndrome is a non specific non neoplastic inflammatory condition of unknown cause that involves extraocular muscles. It is usually confined to the orbit but extraorbital involvement can occur. Ocular manifestations of orbital pseudotumour may include periorbital oedema erythema proptosis ptosis diplopia and pain with eye movements. Here is the case report of a 45 year old female presenting with a bilateral painful orbitopathy with a left lateral rectus palsy. On imaging the aetiology was identified as an infiltrative mass involving left lateral and superior rectus lacrimal gland and cavernous sinus. The workup for differential diagnoses and associations of IOIS ruled out other etiologies and the patient was started on steroids. She showed marked improvement and diplopia improved in a few days. Follow up MRI done 8 weeks later showed marked resolution of the findings. We wish to present the case as the presentation of IOIS as bilateral orbitopathy and myositis with an intracranial extension is not commonly seen.
Case Series of three patients with Scleroderma Polymyositis overlap(SSc-PM) with one year follow up
Shingare Vilas Changde, Rahul Chakor, Haresh Bharote, Nishikant Eklar
T.N.M.C and B.Y.L. Nair Charitable Hospital, Mumbai, Maharashtra, India
Introduction: Myositis in Scleroderma (SSc) patients resembles clinical and biologic features of Polymyositis (PM) hence the term Scleroderma/Polymyositis overlap (SSc-PM). We report three cases of SSc-PM with one year follow up from our hospital. Case 1: 27 years old male patient presented with history of upsquating difficulty since 2 yearsdifficulty in lifting arm overhead since 2 years and swallowing difficulty since 3 months. On examination skin thickening was present. Proximal muscle weakness was present in upper and lower limb(4-/5). CPK was 2053. Serum ANA was positive with nucleolar pattern. EMG and Muscle biopsy was suggestive of polymyositis. Case 2: 31 years old female patient presented with history of dropping of head since 8 month upsquating difficulty since 6 month and difficulty in lifting arm overhead since 4 months. On examination skin thickening was present. Proximal muscle weakness was present in upper(4-/5) and lower limb(3/5). CPK was 900. Serum ANA was positive with speckled pattern. EMG and Muscle biopsy was suggestive of polymyositis. Case 3: 32 years old female patient presented with history upsquating difficulty since 2 1/2 years and difficulty in lifting arm overhead since 2 years. On examination skin thickening was present. Proximal muscle weakness was present in upper and lower limb(4-/5). CPK was 3457. Serum ANA was negative. EMG and Muscle biopsy was suggestive of polymyositis. Discussion: All the three patients fulfilled the criteria for diagnosis of SSc-PM overlap. Patients were started with steroid and immunosuppressant therapy and improved with therapy. At one year follow up all patients are in remission on oral steroids and immunosuppressant. Conclusion: SSc-PM patients usually present with symptoms of Polymyositis. Cutaneous features of Sclroderma should be looked at in all patients with Polymyositis. Prognosis is generally good with steroid and immunosuppressant therapy.
Poster Session 03, Friday, October 02, 2015,
11: 00 AM - 01:00 PM
Dr. Vimla Vermani Hall (Hall D):
3rd Nerve Palsy: An Unusual Cause
Rameshwar Nath Chaurasia
Institute of Medical Sciences BHU Varanasi, Uttar Pradesh, India
Objective: Neurological manifestations of scorpion sting are common in southwest United States and Mexico. However neurological symptoms are uncommon presentations of scorpion sting in the Indian subcontinent. We are reporting a rare case of isolated 3rd nerve palsy secondary to haemorrhagic stroke following scorpion sting. Case history: A 30 year old gentleman presented without any history of medical illness had sting on left 2nd toe by black scorpion while working in the fields followed by severe local pain without oedema with vomiting and generalized tonic clonic seizures 8-10 hours after. On examination: His blood pressure was 180/90 mm of Hg without any rash or signs of bleeding. Patient was delirious and only focal neurological deficit was right third nerve palsy. Investigation: CT head showed right parietooccipital haemorrrhage. BT CT PT APTT and platelet count was normal. Patient was investigated and ruled out for other possible causes of stroke. MRI brain was performed which solved our clinical query of cranial nerve palsy. Discussion: Among the 86 species of scorpions in India Mesobuthus tamulus and Palamneus are of medical importance. Central nervous system complications are very rare and may present in either of two forms both of which are associated with high mortality rates.1) Encephalopathy and 2) Stroke. Almost all cases of sting presented with hemiparesis as neurological deficit in contrast our case presented with right 3rd nerve palsy only. Though uncommon neurological manifestations in the form of ophthalmoparesis can be seen in scorpion sting. Results and Conclusion: Patient was managed conservatively with antiepileptic and prazosine. His sensorium improved over a period of 10 days but right 3rd nerve palsy took 3 month to improve. The suspicion of neurological complications has to be considered and early diagnosis can offer a better prognosis to the patient.
The utility of suggestion techniques in psychogenic nonepileptic seizures: A comparative interventional study
Prasanth. S.R, Shaji. C.V, Kabeer. K.A, Ram Mohan, Parvathy. G, Suraj Menon, Meenakumari
TD M-edical College, Alappuzha, India
Background: Psychogenic nonepileptic seizures (PNES) are paroxysmal events that are often misdiagnosed as epilepsy and leads to the unnecessary use of antiepileptic drugs. The usefulness of suggestion techniques in 56 patients with PNES and their clinical profile is reported here. Methods: All patients presenting with clinically suspected PNES, aged 12 years or more, during the period September 2013 - March 2015 were included. Those with coexisting epilepsy were excluded. Data including age and gender distribution, semiology of episodes and any comorbid psychiatric illness were collected. Detailed neurological examination and mental status examination was done. All underwent 19 channel digital video electroencephalography (EEG) of 40 minutes duration. Out of 56, every alternate patient (n=28) was allotted to suggestion group, who were given simple suggestions that seizure may occur during recording. Any abnormal motor activity if found were reinforced by further suggestions. The other group (n=28) was not given any suggestions. Results: Out of 56 patients 44(78.5%) were females,34 (60.7%) were in the age group 12-20 yrs. 24 patients (42.8%) were already receiving antiepileptic drugs. Main psychiatric comorbidities were depressive disorder in 48 (85.7%),anxiety disorder in 37(66%) and personality disorders in 21(37.5%). In the suggestion group, 19 of the 28 patients (67.8%) had events that were identified as non-epileptic seizures(without any EEG correlate) and confirmed as habitual attack by eye witnesses. Only 6 of the 28 patients (21.4%) in the 'no-suggestion' group had habitual attacks. This was statistically significant difference (p value 0.001) with an odds ratio of 7.714. Conclusions: A short outpatient video EEG record is often sufficient to diagnose PNES, if supplemented with proper suggestion techniques, which would otherwise require prolonged inpatient video EEG recording.
Clinico-Radiological Case Series of Varied Presentation of Vitamin B 12 Deficiency.
Akash Awati, L. Krishnamurthy, Prashant
S. S. Institute of Medical Sciences and Research, Davangere, Karnataka, India
Abstract body not submitted
Speech Signal Analysis and Pattern Recognition in Diagnosis of Dysarthria
Minu George, Anand Kumar, C Santhosh Kumar, John Amose
Amrita Institute of Medical Science, Kochi, Kerala, India
Background: Dysarthria refers to a group of speech disorders resulting from disturbances in muscular control over the speech mechanism due to damage of the central or peripheral nervous system. There is wide subjective variability in assessment of dysarthria between different clinicians. We tried to identify a pattern among types of dysarthria by acoustic analysis to prevent this inter subject variability. Objectives: 1)Pattern recognition among types of dysarthria with software tool and to compare with normal subjects.2)To assess the severity of dysarthria with software tool. Materials and Methods: Speech of 70 subjects were recorded both normal subjects and the dysarthric patients who attended the outpatient / admitted in AIMS. Speech waveforms were analysed using Praat and MATHLAB tool kit. The pitch contour formant variation speech duration of the extracted graphs were analysed. Results: Study population included 25 normals and 45 dysarthric patients. Dysarthric subjects included 24 with extrapyramidal dysarthria14 with spastic dysarthria and 7 with Ataxic dysarthria. Analysis of pitch of the study population showed a specific pattern in each type. F0 jitter was found in spastic dysarthria pitch break with ataxic dysarthria and pitch monotonicity with extrapyramidal dysarthria. By pattern recognition we identified 19 cases in which one or more recognized patterns coexisted. There was a significant correlation between the severity of dysarthria and formant range. Conclusion: Specific patterns were identified and this software tool will help clinicians in better way to identify types of dysarthria and could prevent intersubject variability. We were able to assess the severity of dysarthria by formant range.
Neurological Complications after Bariatric Surgery-A Case Series
Dr. Vasantrao Pawar Medical College, Nashik, Maharashtra, India
Background: Bariatric surgery (BS) is widely accepted as first line therapy for morbid obesity. The incidence of BS is steadily increasing and neurological complications are noted in up to 5% in the post operative course. Several acute and long term complications have been identified. The commonest neurological complications are peripheral neuropathies and less common are encephalopathy myeloneuropathy radiculoneuropathy and Parkinsonism. Objective: To report various neurological complications after bariatric surgery. Cases: Patient profile-Pt 1-51/FHT wt loss-22kg c/o-Muscle cramps and burning paresthesia in Legs EMG/NCS-Axonal SM neuropathy. Normal S b12 S vit D3 Treatment-Pregabalin Multivit+ mineral supplementF/U-2 yrs-Persistent mild symptomsPt 2- 45/F DM HT wt loss-16kg c/o-Symmetrical Slowness and tremers and brdykinesia MRI Brain-Normal TRODAT scan not done Normal S b12 S vit D3 treatment-L-Dopa+Carbidopa Multivit+ mineral supplement F/u 2yrsSymmetrical PDPt 3-53/MDM HT Myasthenia Gravis wt loss-24kg c/o-Worsening of Myasthenia gravis Anti Ach R ab raised EMG/NCS- widespread axonal Radiculo-Neuropathy RNS+MRI Spine- myelomalacia at cervical spine-Spondylotic myelopathy Normal S b12 S vit D3 Treatment-IVIG+IV steroids Ventilation Multivit+ mineral supplement F/u-Death within 3 mths of surgery Discussion: Various central and peripheral neurological complications are noted after bariatric surgery. Partial response and improvement was seen in two patients after treatment while one patient continued to deteriorate despite all efforts and succumbed.
Non Compressive Myelopathies: Etiological Spectrum in a Tertiary Care Hospital
Suvorit Bhowmick, Ashok Kumar Kayal, Munindra Goswami, Marami Das, Lakshya Jyoti Basumatary, Papori Borah
Gauhati Medical College, Guwahati, Assam, India
Background: In a resource limited country like India non compressive myelopathies are rarely investigated thoroughly. Objective: To study the etiologic spectrum of non compressive myelopathies in a tertiary care hospital. Methods: In an observational study carried out in Neurology Department of xxx Medical College from August 2013 to May 2015 patients of non compressive myelopathies were evaluated with MRI spine (brain in selected cases) hemogram blood biochemistries thyroid profile urinalysis chest x-ray CSF analysis and evoked potential studies. Depending on the pattern of involvement further CSF investigations were as follows: IgG index OCBs ELISA or PCR for viruses (VZV HSV CMV EBV enteroviruses JE) ADA and VDRL. Serum investigations included ELISA for HTLV-1 HIV-1/2 and borrelia burgdorferi VDRL and TPHA. Immunological profile included NMO-IgG anti-TPO antibody ANA anti-dsDNA anti-Smith anti-Ro anti-LA anti-tTG and ACE levels. Ultrasound abdomen CT thorax/abdomen endoscopy and bone marrow analysis were done if paraneoplastic etiology was suspected. Results: There were total 125 patients. Median age was 34.5 years. Male: female ratio was 1.2: 1. Etiological spectrum included idiopathic transverse myelitis infectious myelitis (VZV EBV HIV JE M tuberculosis T pallidum C neoformans T gondii) immune mediated myelopathies (MS NMO SLE sarcoidosis Anti-TPO) vitamin B12 deficiency folate deficiency liver disease vascular myelopathies radiation and electrocution injury. Conclusion: We were able to find underlying etiologic agent in 67.2% of our patients. We emphasize comprehensive evaluation of non-compressive myelopathies as they affect many patients who are at the prime of their lives and cause significant morbidity.
An audit of acute symptomatic seizures from tertiary care centre from South India
Jignesh Prajapati, Gopal Krishna Dash
Narayana Hrudayalaya Multispeciality Hospital, Bangalore, Karnataka, India
Background: Very few studies from India have looked into the out come of acute symptomatic seizures (ASS) and audited the patients with ASS. Objective: To audit the etiology EEG finding and outcome of patients with ASS. Material and methods: We prospectively recruited patients with ASS between Dec 2014 till May 2015. Appropriate statistical methods were applied. Results: Seventy five patients were recruited for the study out of which 52(69.3%) were males. Most of the patients had generalized convulsive seizures (65 86.7%). None of the patients had aura. Most common etiology for ASS were cerebrovascular events (stroke hypertensive encephalopathy cerebral hyperperfusion syndrome) seen in 42(56%) patients. Other etiology were infective (21 28%) metabolic (6 8%) tumor (2 2.6%). EEG showed definite spike wave discharges in 7 (9.3%) patients. EEG was normal in 4 (5.3%) patients. Glasgow coma scale (GCS) at entry predicted the out come at discharge (p< 0.0001). Conclusion: Cerebrovascular events are the most common cause of acute symptomatic seizures. GCS at entry predicted the out come at discharge.
Niemann-Pick Disease Type C -A Case Report
Stanley Medical CollegeChennai, Tamil Nadu, India
Introduction: Niemann-Pick disease TypeC(NPD C)a neurovisceral lipidoses is an autosomal recessive disorder. Incidence is 1 in 150000. Case Report: 5 year old boy born for consanguineous parents with uneventful birth history started having frequent fallsand progressive difficulty in walking since 1 1 /2 yearsepisodes of GTCS since 1 year and involuntary movements in upper limbs since 3 months. He had normal milestones except for delayed language development. Family history was negative. On examinationchild was able to obey simple commands. Fundus was normal. He had upward gaze palsystriatal toe involuntary movements in upper limbs and was able to walk with support of others. Other systems examinationnormal. Investigations MRI Brain showed subtle T2hyperintensity in periventricular white matter. USG Abdomen Smear for acanthocyte and Wilson disease workup were negative. Bone marrow aspiration revealed Sea blue histiocyte(SBH) suggestive of NPD C. Discussion: NPD C present with developmental delay or regression cataplexyseizureataxiaextrapyramidal featuresvertical gaze palsy and hepatosplenomegaly. Sphingomyelinase activity in WBC is normal in NPD C unlike in other types. Mere presence of SBH is not specific for NPD Cbut in the appropriate clinical setting its presence leaves no doubt to diagnosis. Diagnostic confirmation is by skin fibroblast cuture and Filipin staining for intralysosomal storage of unesterified cholesterol(not done in this case). Conclusion: This case is presented for its rarity. Vertical gaze palsy in a child with extrapyramidal featuresone should consider the possibility of NPD C.
Poster Session 04, Friday, October 02, 2015,
11: 00 AM - 01:00 PM
Dr. Vimla Vermani Hall (Hall D):
A Rare and Interesting Case of Generalized Dystonia Following Suspected Snakebite Treated with Antisnake Venom
Sowmya M,B Nataraju, Sreekantaswamy, Nischal Reddy, Anantha G, GurucharanAdoor, Srinivas Raju
VIMS &RC Bengaluru, Bengaluru, Karnataka, India
Master X a 13 yr old boy form West Bengal India had a suspected snake bite on the dorsum of his right hand in May 2014 when he was fishing in a fresh water pond. He developed pain immediately at the site of bite and an hour laterlost consciousness with frothing from the mouth & swelling of whole of the right upper limb. He was hospitalized and received emergency treatment which included about 20 vials of polyvalent antisnake venom. He regained consciousness a day later and over 2-3 daysaround 5th day of his illness became restless irritable was bedridden and developed sudden onset rapidly progressive generalized dystonic movements of the whole body including head and trunk which progressed over 1-2 days and remained constant thereon with intermittent spontaneous exacerbation of dystonic movement causing him pain which made him cry out periodically with irritability and spasticity of both lower limbs. His trunk and neck was bent towards the left with writhing movement of the whole body. MRI Brain done on 6th day of the illness showed bilateral striatal T2 & FLAIR hyperintensities. He was referred to us for further management in view of non improvement of symptoms about a month after the suspected snake bite. Here on evaluation at our institution a diagnosis of generalized dystonia secondary to bilateral striatal necrosis due to possible snake bite was made. The possible pathophysiological mechanism suspected were :1. ? Hypoxic ischemic injury 2.? Snake venom toxin induced? Immunological reaction to antisnake venom? immunological reaction secondary to infection of wound with organism like streptococcus Mycoplasma pneumonia (which are known to cause bilateral striatal pathology). He was treated with oral Prednisone 10 mg TDS for 3 weeks which was gradually tapered over 2 mthsTrihexyphenidyl Baclofen & diazepam for the dystonia Oxcarbazepine for suspected neuralgic pain. A week after starting treatment patient gradually started improving initially with decrease in restlessness & crying and a little later dystonia & spasticity of both lower limbs also reduced and on follow up after 3 mths he had remarkably improved wherein he was able to walk independently dystonic posturing remaining only at the toes bilaterally at the metatarsophalyngeal joints with ulcer over the right hand for which he later underwent plastic surgery. MRI brain was repeated on follow up in April 2015 which showed definitely reduced intensity of bilateral striatal necrosis. He was cheerful & smiling & is planning to go back to school soon. To our knowledge this is the first reported case of generalized dystonia following suspected snake bite which improved completely with steroids and other supportive therapy. As the patient improved in his dystonia almost completely &hyperintensity of the striatum bilaterally definitely became much less an hypoxic ischemic basis might be unlikely & an immunological pathogenesis might be a likely possibility.
Case Series of Hemidystonia-Hemiatrophy Syndrome
Harisudan S,Shankar Balakrishnan, Asir J, Ramakrishnan V, Chandramouleeswaran V, Lakshminarasimhan R, Bhanu K
Madras Medical College, Chennai, Tamilnadu, India
Introduction: Dystonia is a common extrapyramidal manifestation seen in association with various neurological and systemic conditions. Hemidystonia has been noted in vascular infective metabolic hereditary drug or toxin induced etiologies. Hemidystonia - Hemiatrophy is a rare inherited disorder presenting with early onset dystonia. This condition is associated with hemiatrophy of the face +/- limbs. Here we present two cases of Hemidystonia-Hemiatrophy syndrome due to its rarity. CaseVignettes: Case1: 24 yr old male born of a non-consanguinous marriage with normal developmental milestones presented with 2years history of insidious onset gradually worsening dystonic posturing involving the right upper and lower limbs. Patient also had hemiatrophy involving the right half of the face and right upper limb. MRI brain showed pachygyria involving the left frontal &frontoparietal region. Patient was treated with clonazepam and trihexyphenidyl and his symptoms improved. Case 2: 18 yr old male born of a non-consanguinous marriage with normal developmental milestones presented with insidious onset of gradually worsening dystonic posturing involving the right upper limb and torticollis for 6 months with 2 episodes of right focal seizures with no generalisation. Patient also had hemiatrophy of right half of the face with apparent right enophthalmos. MRI brain showed evidence of pachygyria involving left frontoparietal area. Patient started on carbamazepine clonazepam &trihexyphenidyl and showed improvement. Conclusion: Hemidystonia-Hemiatrophy syndrome is a rare cause of hemidystonia invariably associated with structural developmental lesions like pachygyria and the diagnosis requires high degree of suspicion.
Sporadic case of Secondary Paroxysmal Dyskinesia/Dystonia.
Priyank Shah, Sudhir Shah,Mayank Patel,Pranavjoshi
NHL Medical CollegeVS Hospital, Ahmedabad, Gujarat
Paroxysmal movement disorders are defined as abnormal involuntary movements that are intermittent or episodic in nature with sudden onset and with no change in consciousness. The episodic involuntary movements may be dystonia chorea ballismus or a complex combination of these hence referred to as paroxysmal dyskinesia. There are primary and secondary causes of Paroxysmal dyskinesia among primary causes are more common. Here we have reported a sporadic case of secondary paroxysmal dyskinesia. 9 years old boy presented with recurrent episodes of difficulty in walking since 5 years and each episode lasting for 1-2 days and precipitated by headache / fever. Lastepisode persistent since 1 month. No other positive history noted. On CNS examination -bilateral foot dystonia on walking with presence of KF ring on slit lamp examination rest of the examination is normal. Evaluation revealed mildly raised SGPT with significant high 24 hours urine copper with liver parenchymal disease in USG abdomen. MRI Brain and spine screening normal. So even in episodic paroxysmal dyskinesia Wilson disease should be consider as a treatable cause.
A Novel Scan in Wilson's Disease
Muthukumaran. S,Lakshminarasimhan. R, Nandakumar. V, Kannan. V, Thamilpavai. N, Sarala. G, Bhanu. K
Introduction: Wilson's disease is an autosomal recessive disease due to an impairment of cellular copper transport presenting with neurological psychiatric and hepatic manifestations either in isolation or in combination. Patients presenting with neurological symptoms may have no clinical evidence of liver involvement. Transient elastography (fibroscan) is a novel noninvasive test to measure the liver inflammation and fibrosis as comparable to liver biopsy. Aims and Objectives: To identify the subclinical hepatic involvement in patients with normal clinical and biochemical hepatic function presenting with neurological Wilson's disease. Materials andMethods: It is a observational study which included ten patients with a diagnosis of neurological Wilson's disease attending department of neurology RGGGH Chennai. Wilson's disease patients with known liver involvement and abnormal Liver function tests were excluded from the study. They were subjected to fibroscan and ultrasound abdomen. Results: In our study significant fibrosis as evidenced by a fibroscan value of more than 7kPa was found in 70% of patients. Among them 30% had a value of more than 14kPa which is suggestive of cirrhosis and USG abdomen showed features of liver cirrhosis in them. MRI Brain showed abnormalities in 30% of patients in the form of T2 and FLAIR hyperintensities in caudate putamen thalamus and midbrain. In this group 66% of patients had a fibroscan value of more than 14kPa. Conclusion: Thus fibroscan helps us to identify hepatic fibrosis and cirrhosis in neurological Wilson disease patients with normal clinical and biochemical hepatic function. Hence all patients with neurological Wilson's disease should be subjected to fibroscan regardless of their clinical hepatic involvement.
Spectrum of Movement Disorders induced by Hyperglycemia
Prabaharan C, Chandramouleeswaran V, Venkatraman K, Ramakrishnan V, Sarala G, Bhanu K
Madras Medical College, Karur, Tamil Nadu, India
Introduction: Hyperglycemia is a risk factor for many neurological problems. Hyperglycemia produces various movement disorders like chorea hemichorea-hemiballismusathetosis and various unclassified movement disorders. These disorders are rarely studied. Aims and Objectives: To study the spectrum of movement disorder induced by hyperglycemia. Materials and Methods: It is an observational study which included 30 inpatients with movement disorder precipitated by hyperglycemia admitted in neurology and medical wards in RGGGH Chennai. Movement disorders in patients with normal blood sugar and movement disorder due to structural and other metabolic causes were excluded from the study. The study period was between November 2014 to June 2015. Results: In our study among 30 patients 28 were known diabetics with poor glycemic control and 2 were newly diagnosed diabetics during the evaluation of movement disorder. The common movement disorders were generalised chorea 4 (13.3%); hemichorea-hemiballismus 16 (53.3%)]; myoclonus 2 (6.6%); perioral dyskinesia 4 (13.3%); hand tremors 2 (6.6%) and other unclassified movements 2 (6.6%). Neuroimaging revealed diabetic striatopathy in 10 patients and rest were normal. The blood sugar levels of all the patients were above 400mg/dl. Perioral dyskinesias were earlier movement to disappear with glycemic control requiring no treatment while patients with hemichorea-hemiballismus needed drug therapy despite glycemic control for recovery. Perioraldyskinesias resolved between 3-6 days and resolution of chorea took longer time ranging from 2 weeks to 4 weeks Conclusion: Routine screening of blood sugar should be done in all patients with movement disorders. Better the glycemic control earlier will be resolution of abnormal movements.
Acute Rigid State - A Case Series
Venkatraman K, Chandramouleeswaran V, Prabaharan C, Ramakrishnan V, Sarala G, Bhanu K
Madras Medical College, Chennai, Tamil Nadu, India
Background: The aetiologies and the management between the acute onset and insidious onset rigidity syndromes vary considerably. It is of great importance to identify them without delay. Aim of The Study: To study the etiological and prognostic factors of cases presenting in acute rigid state. Methods and Materials: It is an observational study which included 16 patients presenting with Symptoms of stiffness of limbs and slowness of activities/speech of less than 2 weeks duration. Patients Aged>50 yearsInsidious onset of symptoms and those with previous use of levodopa were excluded. Study period was between October 2014 and April 2015 Results: We identified 16 cases. Of these 14 cases had etiologies involving basal ganglia and its connection and 2 cases were due to tetanus involving the inhibitory neurons at the spinal cord. Among the cases with CNS involvement 6 cases had infectiousetiology (JE Virus(3) HSV(1)Measles(1)-post infectious).4 cases were due to structural pathology(ischemic stroke (2)hydrocephalus(1)extrapontinemyelinolysis(1). 2 cases were Neuroleptic Malignant Syndrome.1 case was reported in SLE. 1 case was psychiatric(catatonia). Mortality and morbidity rates were 25% and 30% respectively. Residual rigidity at 6 months was 31%. The mortality and morbidity was high among patients with delay in consultation/diagnosis(75% and 60% respectively) Conclusion: Early Diagnosis of acute rigid state is necessary as the mortality rates Is high. The knowledge on the possible etiological factors helps to optimize patient management and helps determine the prognosis.
Interesting cases of Neuromyotonia
Rushikesh Deshpande,Sangeeta Ravat, Juber D. Shaikh, Nilesh Nagargoje
Seth GSMC and Kemhmumbai, Mumbai, Maharashtra, India
Introduction: Issacs described a condition of "Continuous muscle fiber activity" or "Armadillo disease". It can present with continuous undulating rhythmic movements most common over thigh and calf muscles. Neuromyotonia with central and autonomic features is called as Morvan's disease. Underlyingneopolasm should be excluded as it can be a paraneoplastic manifestation. Methods: We describe two cases of neuromyotonia. Case 1: 53 year old male presented with 4 week history of calf pain muscle twitching and difficulty in walking. On exam patient had a normal power but continuous bag of worms like fasciculations. Case 2: 19 year old male with 3 month history of calf pains twitching movements in calves and arms with autonomic features. On examination patient had a significant postural hypotension and continuous fasciculations. EMG in both patients demonstrated repetitive burst of quadruplets and multiplets with high intra burst frequency suggestive of neuromyotonia. CSF exam and whole body PET scan was normal. First patient was detected positive for antiVGKC antibody second patient was negative. Both patients received course of IVIG and IV methylprednisolone and oral phenytoin. Both patients showed significant improvement in symptoms. Conclusion: Neuromyotonia is usually mediated by antiVGKC antibody. Immunotherapy is required in most cases. Underlying neoplasm should be removed as a curative therapy.
A Rare Neurometabolic Disorder: PKAN- A Case Report
Raja K S, S. Gobinathan, S. Sakthi Velayutham, K. Malcolm Jeyaraj, P.R. Sowmini, K. Karunakaran, S. Ravindran, P. Sampath Kumar, A. Vignesh
Govt. Stanley Medical College, Chennai, Tamilnadu, India
A 15 year old boy born to third degree consanguineous parents presented with history of blurring of vision more on dim light since 5 years of age. Patient had abnormal posturing of both hands while eating and writing for the past 1 year. Patient also had speech difficulty for the past 1 year. His scholastic performance was also worsening for the past two years. On cranial nerves examination patient had restricted peripheral visual field and feature of retinitis pigmentosa on fundus examination. On motor system examination there was no wasting (or) weakness. Deep tendon reflexes and bilateral plantar reflexes were normal. But patient had action dystonia in both hands and orofaciolingual dystonia. His sensory and cerebellar systems examination were normal. Routine blood investigations peripheral smear study seurmceruloplasmin and serum ferritin were within normal limit. MRI brain showed Eye of the Tiger sign a central region of hyperintensity surrounded by a rim of hypointensity on T2 weighted image of globus pallidum suggestive of PKAN. There are three forms of PKAN: Classic atypical and intermediate form. Classic PKAN develops in the first 10 years of life and more rapid progression of symptoms. In atypical form of PKAN usually symptoms starts after ten years and progresses more slowly. In our patient though symptom was started in five years of age it has been slowly progressive for the past ten years fits him under intermediate category which is rare among the three forms of PKAN. This case is presented here for its rarity.
Poster Session 05, Friday, October 02, 2015,
02: 00 PM - 04:00 PM
Dr. Vimla Vermani Hall (Hall D): Headache
Unusual Headache Patterns in Behcet Disease
Prashant Kumar, Kuljeet Singh Anand, Jyoti Garg
PGIMER & Dr. RML Hospital, New Delhi, India
Introduction: Behcet disease is a systemic vasculitides characterized by recurrent oro-genital ulcerations uveitis and positive pathergy test. Neurological involvement occurs in 5-40% of patients in the form of recurrent meningoencephalitis veno-occlusive disease and brain stem involvement. We are reporting two cases of Behcet disease one with severe headache with meningismus and other with thunderclap headache which has not been described in literature. Case 1: A 32-year-old non-diabetic normotensive male presented with recurrent oral and genital ulcers since 3 months. Severe holocranial headache without vomiting and unresponsive to analgesics since 15 days. Headache was associated with severe neck pain and restricted neck movement. On examination he had apathous ulcer scrotal ulcer erythema nodosum and severe neck stiffness. Fundus was normal and there was no focal neurological deficit. Routine investigations and X-ray of cervical spine were normal. CSF analysis showed clear fluid no cells protein 37mg% and sugar 62mg%. Pathergy skin test was positive. MRI and MRV head were normal. His headache responded to IV dexamethasone. Case 2: A 43-year-old non diabetic hypertensive male a known case of Behcet disease presented with thunderclap headache. His vitals and sensorium were normal and there was no focal neurological deficit at the onset. His imaging showed bleed in the supracellar region. Subsequently he developed features of adrenal insufficiency which was managed with high dose IV steroid followed by oral steroid. His headache improved gradually. In follow up imaging hematoma resolved with no evidence of pituitary adenoma. Conclusion: In Behcet disease unusual headache pattern can occur apart from commonly described TTH migraine headache headache due to meningoencephalitis or due to dural venous sinus thrombosis.
Clinical and Temporal Profile of Sexual headache
M. Abid,S. Balasubramanian, R. Subramanian, N. Ramakrishnan, K. Bhanu
Madras Institute of Neurology, MMC, Chennai, Tamil Nadu, India
Primary headaches associated with sexual activity. Aim: To find out the prevalence temporal profile and causes for sexual headache in patients attending headache clinic. Methods: A cross sectional study among 100 patients between a 20-45 yrs interviewed 20 patients who had symptomatic experience of sexual headache as perInternational Headache Society diagnostic criteria for primary sex headaches were evaluated. Results and Discussions: Among the patients who had sexual headache 65% were Male 35% were Female Age of distribution of sexual headaches 60% were between 20-30 yrs. Onset of sexual headache was 25 % during foreplay 30 % at mounting sexual excitement 35 % at the moment of orgasm 10%occurs after coitus. Duration of sexual headache was 25 % had less than a minute 55 % one minute to 24 hrs 20% had more than 72 hrs. Site of orgin of sexual headache 45 % had occipital orgin. Quality of pain during headache 45 % had throbbing pain 30 % had pulsating pain 20% had expoding pain 50 % grade 2 disability on assessment midas score. 55 % felt better after four weeks of therapy with b blocker. MRI BRAIN FINDINGS: 2 patients had AVM1 had cavernous angioma 1 had chari type 1 malformation. Conclusions: In our study we found that sexual headache had male predominance majority were between 20-30 yrs of age with of throbbing in quality of pain at the moment of orgasm lasting between 1 min to 24 hrs more in the occipital region with grade 2 disability on MIDAS score. 55 % patients feeling symptomatically better with therapy.
Clinical Profile of Cavernous Sinus Syndrome (CSS) in a Tertiary Care Centre in India
Sanat Bhatka, Manoj Goyal, Paramjeet Singh, K. K Mukherjee, Vivek Lal
Introduction: CSS is one the commonest causes of painful ophthalmoplegia worldwide common causes of which include bacterial or fungal infections non-infectious inflammation vascular lesions and neoplasms. There is paucity of data regarding the clinical profile of CSS in Indian population which is supposed to be different from western world due to high prevalence of infections in Indian population. Objective: To analyse the clinical profile of CSS in a North Indian tertiary care hospital. Materials and Methods: It was an observational study. Inclusion criteria were involvement of 2 or more of the third fourth fifth (V1 V2) or sixth cranial nerves or involvement of only 1 of them + neuroimaging-confirmed cavernous sinus lesion. All patients were subjected to routine and specific investigations (CSF CEMRI Brain FESS biopsy). The patients were accordingly subjected to medical or surgical treatment and followed up for 3 months. Results: 73 patients have been enrolled in the study. 47 of these are males and 26 are females. Amongst causesthe commonest aetiology was Neoplasms (28.8%) fungal infections (24.6%) and tolosa hunt syndrome (23.2%) vascular etiologies (6.8%) while other causes like hypertrophic pachymeningitis Sarcoidosis and wageners granulomatosis made up the list (16.4%). Diplopia was the commonest symptom while 6th nerve was the commonest nerve involved. Discussion and Conclusions: Neoplasms continue to be the commonest cause of CSS. However treatable causes(infectionsTHS) make up around 55% of these causes especially if detected early.
Outcome of 10 patients with Idiopathic Intracranial Hypertension "Role of therapeutic lumbar puncture"
Sritharan, Jason Ambrose, Chezhian, Birla Pavalam, Justin, Ganesan, Manivannan
Madurai Medical College, Madurai, Tamil Nadu, India
We followed up 10 patients with idiopathic intracranial hypertension for a period of 1 year. The mean CSF Pressure was 31.7cm (range 26cm to 45cm). Four of our patients improved symptomatically with initial medical management and their visual fields and acuity did not show any deterioration. Two of our patients had persistent headache with medical management but there was no deterioration in visual fields or acuity. Both of them remained stable on symptomatic management and had good resolution of papilloedema. The remaining four had worsening visual acuity with field constriction despite medical therapy. For these patients we did therapeutic lumbar punctures with removal of 20ml of CSF. Out of four patients who had therapeutic LP three patients improved after 3 therapeutic lumbar punctures and were continued on medical management. All three who improved with therapeutic LP remain clinically stable on outpatient follow up. One patient continued to have headache with papilloedema and visual deterioration even after 3 therapeutic lumbar punctures. She was referred to neurosurgery department for lumboperitoneal shunting. Therapeutic lumbar puncture is generally viewed as a transient measure to stabiles the patient as a bridge to a shunting procedure. But our findings suggest that therapeutic lumbar puncture may provide longer lasting benefits. This deserves further study.
Sympathetic Skin Response in Patients with Migraine
M. Mohamed Kilji, A.C. Somasundaram, N. Shanmugasundarm, S. Balasubramanian, K. Bhanu
Institute of Neurology, Chennai, Tamil Nadu, India
Background: Autonomic system dysfunction is well noted in patients with migraine. Herein we study the changes in sympathetic skin response in migraine patients to assess the integrity of autonomic nervous system and its dysfunction. Aim: To compare Sympathetic Skin Response(SSR) in patients with migraine during ictal periods and controls. Materials And Methods: 20 Patients [2 male (10%) 18 female (90%)] and 10 controls were recruited from Madras Institute of Neurology Chennai and were subjected to Sympathetic Skin Response on both sides of the upper and lower limbs of all subjects. Results: In all subjects responses were obtained. Patients with migraine during ictal period had longer latency in SSR (14 out of 20 patients 70%) when compared to control group. These changes were statistically significant (<0.05). Conclusion: Migraine patients showed significantly longer SSR latency than controls and it can suggest that sympathetic hypofunction occurred in patients with migraine during ictal period.
A Polycythemia Vera presenting with Intracerebral Complicated by Deep Venous Thrombosis- A Case Report
Mitalee Kar, Hari Radhakrishna, Bimal Padhy, Sailesh Badole, Sandeep
Care Hospitals Hyderabad, Hyderabad, Telangana, India
Introduction: Polycythemia is a laboratory finding with increased number of RBC in the peripheral blood. It is present if hematocrit (HCT) is >48 or >52 percent; or when the hemoglobin concentration (HGB) is >16.5 or >18.5 g/dL in women and men respectively. Objective: In this case report we have discussed how we managed polycythemia and tackled Deep Venous thrombosis (DVT) in presence of acute intracerebral hemorrhage(ICH). Methods and Results: A 55 years old male nonsmoker patient presented with ICH with intraventricular extension & subarachnoid hemorrhage. He underwent emergency decompressive craniotomy and evacuation of hematoma. He was on DVT prophylaxis (initially in view of large ICH heparine or LMWH was avoided and managed with DVT stockings and intermittent pneumatic compression devices). He was evaluated and found to have Polycythemia Vera (low serum erythropoietin JAK2V617F mutation positive and bone marrow suggestive of hypercellularity with panmyelosis) managed with phlebotomy & Tab. hydroxyurea. Subsequently (after 1 week of admission) he developed left LL & left UL DVT and managed with LMWH. After next 5 days he was found to have developed DVT also in right LL. He was continued on LMWH which was subsequently overlapped and replaced with oral anticoagulant. Repeat imaging was suggestive of no recurrence of bleed. Conclusions: Polycythemia may present as thrombotic or hemorrhagic complications. Management of patient varies case to case and needs to be modified according to clinical situation.
Role of Transcranial Doppler Study in Migraine with and without Aura
Pradeep Kakarala, A. Gopi Krishna, Vindhya Tulasi, U. Aruna Kumari, T. Sateesh Kumar, S. Gopi, G. Butchi Raju
Andhra Medical College King George Hospital, Visakhapatnam, Andhra Pradesh, India
Background: Migraine is a common disabling brain disorder whose key manifestations are recurrent attacks of unilateral headache and interictal hypersensitivity to sensory stimuli. Transcranial Doppler (TCD) is a useful non-invasive modality in evaluation of blood flow velocities in migraine thus contributing to its pathophysiology. Aims and Objectives: To use TCD to determine if patients with migraine with or without aura have any ictal or interictal hemodynamic abnormalities compared with controls. Materials and Methods: 50 patients who presented to Neurology OPD with headache fulfilling ICHD criteria for migraine with or without aura were studied. The Sonara Tek Transcranial Doppler machine from Viasys Neurocare was used. 2 MHz transducer was used to study MCAACA and PCA blood flow velocities on both sides sequentially. Results: On comparing mean of mean velocities and peak velocities of MCA ACAPCA in subjects with migraine in ictal period with those of controls right MCA mean velocities and peak velocities were lower (p value<0.03). On comparing the velocities of right hemicranial headache group with controls MCA mean velocities were found to be significantly low on right side (p value<0.04). No significant difference in mean or peak velocities of other arteries on both sides and also in interictal studies. Conclusion: Reduction of MCA flow velocities was observed in migraine without aura group and on right side in right hemicranial headache group a finding that supports arterial vasodilation theory of migraine. This study shows TCD sonography corroborates with the pathophysiological hemodynamic abnormalities seen in ictal phase of migraine patients.
Poster Session 06, Friday, October 02, 2015,
02: 00 PM - 04:00 PM
Dr. Vimla Vermani Hall (Hall D): Dementia
Balint Syndrome- A Case Report
Muthu Kumar, Thirumalai Rajan, Justin, Ganesan, Manivannan, Sritharan
Madurai Medical College, Madurai, Tamil Nadu, India
Balint syndrome is a condition resulting most often from damage bilaterallyto the parieto-occipital cortex. The syndrome is manifest clinically as a triad of symptoms including optic ataxia paralysis of gaze and spatial disorder of attention. Case Vignette: 36 YEAR old male patient presented to us with difficulty in carrying out grillwelding work with precision as before for the past 8 months.8 months back patient had sudden onset of difficulty in localizing welding junctiondifficulty in plugging in a 3 socket plug pointdifficulty in keeping the paste exactly in the tooth brush. He also complained that his body is distorted or tilted to the right side. No other positive history. The above problem was of acute onset and persisting in nature in the past history3 years before the onset of this problem patient had sudden onset of abnormal sensation over left hand with unintentional deviation of his left hand at that time.. Personal history revealed that he is a smoker alcoholic and also a polysubstance abuser. On examination patient had constructionalapraxiaopticataxiasimultaneous agnosiadyscalculia and alexia. Other lobar functions cranialspinomotor systemsensory and cerebellar function were normal. Routine investigations including blood sugar renal function test LFT serum electrolytes lipid profilecomplete hemogram and thyroid profile were normal. ECG ECHO were normal. HIV ELISAVDRLANA were negative. MRI brain with MRA/MRV showed bilateral parieto-occipital infarct. Carotid and vertebral arterial Doppler done showed normal study. Patient was treated with antiplatelets statins and ather supportive measures. Conclusion: Our patient has got classical features of balint syndrome of optic ataxia oculomotor apraxia and simultaneous agnosia. This is often associated with bilateral damage to parietooccipital cotex as in our case. The case is presented for its rarity.
Bilingualism Delays Age of Onset of Mild Cognitive Impairment
Jagadeesh Babu Kovvuru,Sireesha Yareeda, Divya Raj Golahatti, Amulya Rajan, Shailaja Mekala, Suvarna Alladi, Subhash Kaul
NIMS, Hyderabad, Telangana, India
Background: Several studies have found that bilingualism delays the onset of dementia. There are fewer attempts to understand its effect on Mild Cognitive Impairment (MCI) a preclinical stage of Alzheimer's dementia. Through this study we attempted to understand the association between bilingualism and MCI in the multilingual context of Hyderabad. Methods: 115 patients with MCI (93 bilinguals) diagnosed according to NIA AA criteria who were evaluated in a memory clinic formed the cohort. Bilinguals and monolinguals in the group were examined on their clinical and demographic profiles and were compared for their age at presentation and age at onset adjusting using general linear model for other possible confounding variables like gender education occupation vascular risk factors. Results: Bilingual MCI patients were found to present 7.2 years later than monolingual MCI patients a difference that was found to be statistically significant after adjusting for other confounding variables including gender education and occupation. Conclusions: This is the largest study so far to examine the effect of bilingualism on MCI. The protective role of bilingualism on cognitive impairment preceding dementia was established suggesting that the protective effect of bilingualism lies along a continuum from normal to pathological states
Study on Clinical Spectrum of Vascular Cognitive Impairment
Vadivel S,K. Bhanu
Institute of Neurology Madras Medical College, Coimbatore, Tamil Nadu, India
Introduction: Vascular dementia is the second most common cause of dementia comprising about 1/3 of all dementia. In India we have increased incidence of stroke and high number of cardiovascular disease burden which have contributed to increasing number of patients with VCI. Aim: To study the clinical spectrum of vascular cognitive impairment. Objectives: To determine the risk factors for VCIοƒ To analyze the clinical spectrum and cognitive profiles in VCI Material and Methods: This is a prospective analytic study. 50 Vascular dementia patients fulfilling NINDS - AIREN criteria (probable and possible) were studied for one year (Sep 13 - Aug 14) at Institute of Neurology Chennai. The clinical spectrum of VCI was assessed by MRI and clinical profile. Cognitive functions were assessed by MOCA Addenbrookes test. The significance of score was obtained by using ANOVA test and post HOC test. Results: The risk factors of VCI such as age sex education duration DM SHT dyslipidemia Coronary artery disease atrial fibrillation smoking alcohol were analyzed by chi square and found that all are clinically and statistically significant. This spectrum is broadly classified into large vessel disease (42%) small vessel disease (32%) strategic infarct (10%) dementia due to intracerebral hemorrhage (8%) and minimal cognitive impairment (8%). The executive functions Visuospatial fluency attention memory are most commonly affected in vascular dementia. Conclusion: In our study hypertension diabetes and CAD stays an important risk factor for VCI. In the spectrum of VCI the most common disorder was found to be large vessel disease(42% ) followed by small vessel disease (32%) strategic infarct (10%) hemorrhagic dementia (8%) and MCI (8%). The executive functions are most commonly affected in vascular dementia compared to other dementia.
Serum Homoscysteine Levels in Alzheimer disease: A case control study.
Vikas Dhikav, Kuljeet Singh Anand, Lokesh Sharma, Neera Sharma.
PGIMER, New Delhi, Delhi.
Introduction: Alzheimer's disease (AD) is by far the most common type of dementia worldwide. Currently role of vascular factors have been increasingly recognized in pathophysiology of AD. Serum homocysteine which is a marker of vascular risk has been under investigation. The present study was undertaken to explore its association in AD. Methodology: This is a case control study (n=65). The diagnosis of AD was made using National Institute of Neurological and Communicative Disorders and Stroke Alzheimer's disease related Disease Association criteria (NINCDS-ARDA). After an overnight fasting blood samples were withdrawn from the selected cases and controls. They were centrifuged and serum was isolated and stored in deep freezer for analysis they were thawed at 4-80 and warmed at room temperature (250). The homocysteine levels were estimated using chemiluminescence immunoassay. Results: In AD group (n=35 M: F=29:7) mean age and duration of illness was 728.1 and 3.12.2 years respectively while in controls (n=30 M: F=24) mean age: 58.68.6 years. The mean MMSE score of AD group was 13.325.6. AD group showed high homocysteine levels and the odds ratio was higher in AD compared to controls (6.5; p-value<0.05 95% confidence interval= 1.79 to 24.069). There was high intra-rater agreement (Cohen's Kappa=0.8) in between two raters who separately analyzed the samples (n=10). Conclusion: AD group had higher serum homocysteine compared to age matched controls highlighting its role in pathophysiology of AD.
Spectrum of Dementia in a Rural Based Medical College
Souvik Dubey,Bitoti Chatterjee, Mahua Dubey, Mrinal Kanti Ghosh, Asim Kumar Mallick, Sandip Pal,
Debal Laha, S.B. Roy, A.K. Senapati, S.K. Das
Background: Most of the studies on dementia in India are from urban areas which constitutes 30% of Indian population. Studies from rural setting are scarce. Objectives: Objectives are to diagnose dementia and its various subtypes among rural patients clinically and radiologically to find out rural scenario of dementia. Methodology: We evaluated subjects with possible cognitive dysfunction from admitted and OPD patients through history and clinical examination including BMSE(validated Bengali Mental State Examination) as screening tool and KCB(Kolkata Cognitive Battery)for various domain involvement and EASI(Everyday Ability Scale for India)for activities of daily living and Geriatric depression scale for depression. MRI brain Blood and relevant biochemical tests were done. Results: A total of 64 patients (M : F- 1.8:1) were diagnosed with dementia. Mean age was 65 years. Among them following distributions were noted based on subtypes and etiologies: vascular-16 degenerative-30 mixed-4 metabolic-3 surgical cause-5 NPH-1 infective-5. Reversible dementia was 14(21.9%) and Young onset dementia (<65 years) was 10 (15.6%). Imaging showed evidence of ischemia in all cases of vascular dementia and in three patients with MD. AD showed focal temporal atrophy 4 diffuse atrophy 3 posterior cortical atrophy 1 and normal 3. FTD patients showed medial frontal and temporal atrophy 5 perisylvian atrophy 1 and left frontal with insular atrophy 1. Two PSP patients had mid brain atrophy. One HD patient had caudate atrophy. MRI also detected secondary causes of dementia. Conclusion: In comparison to predominant vascular dementia in central India study and degenerative dementia in Western India study our study shows vascular dementia in 25% and degenerative dementia in 46%. Result indicates referral bias like other hospital based studies. Brain imaging helped in correlating diagnosis. Overall we noticed no definite differences from urban studies.
Profile of Metabolic Encephalopathy Patients in a Tertiary Care Hospital in South India
Santhosh Perumal, Meenakshisundaram, Velmurugendran, Shankar, Prabash
Sri Ramachandra University, Chennai, Tamil Nadu, India
Introduction: Metabolic encephalopathyare common among patients in the critical care unit. Dyselectrolytemia septic hepatic and hypoglycemic encephalopathy are most frequently seen. They produce global neurological dysfunction ranging from lethargy or mild confusion to coma. Metabolic encephalopathies must be distinguished from other conditions such as structural brain lesions infections of the central nervous system or drug reactions. Aim: To study the clinical profile of patients diagnosed with metabolic encephalopathy in a tertiary care hospital. Materials and Methods: This is a retrospective study. 52 patients who were diagnosed with metabolic encephalopathy in Sri Ramachandra medical centre from April 2013 to April 2015 were included in the study. The age range of the patients was 18 years to 90 years. Neuroimaging and CSF analysis was done in all patients to rule out structural brain lesions and CNS infections respectively. Results: Out of 52 patients 80.7% presented with altered sensorium and drowsiness 7.6% presented with seizures and 11.5% presented with unresponsiveness. Hyponatremia was the most common cause of metabolic encephalopathy seen in 42.3% patients followed by septic encephalopathy in 13.4 % patients Hashimoto's in 11.5% patients hyperglycemia in 3.8% patients hypoglycemia in 3.8 % steroid responsive encephalopathy in 3.8% posterior reversible encephalopathy in 3.8% hepatic encephalopathy in 5.8% patients wernicke's in 3.8% and hypovolemic encephalopathy in 1.9 % patients. Mortality percentage was 5.7%. Conclusion: The most common presenting complaints were altered sensorium and giddiness and the most common cause of metabolic encephalopathy was hyponatremia.
Cross Modal Activation of Language Function in Speech Therapy
Apoorva Pauranik,Pinky Singh
MGM Medical College, Indore, Madhya Pradesh, India
Background: Neural multi-functionality and tightly interwoven nature of language and action contribute to recovery from aphasia. Aim: To document clinical utility of the augmentative role of cross-modal transfer of linguistic skills through focused and massed practice of preserved skills in aphasia rehabilitation. Methodology: A single case report comparing patient's stable and plateaued performance in a few specific task before and after a focused intervention. AD 65 year right handed male 17 years of education one year post stroke right hemiplegia with transcortical motor aphasia had following baseline scores on Western Aphasia Battery subtests: spontaneous speech: 5/20 reading subtest: 30/100 writing subtest: 0/100 (completely denied by case due to right hemiplegia). He performed well on written word stimulus-object/picture choice matching and vice-versa task. Treatment approach was based on massed practice in short time Result and Discussion: Participant's performance improved significantly in all domains after 22 sessions with frequency of thrice/week. Post intervention scores on WAB subtests were: spontaneous speech: 10/20 reading subtest: 50/100 writing subtest: 52/100. Treatment also included facilitation of writing by right paretic hand with passive support which was withdrawn gradually. Generalisation effect of writing task was observed on reading along with cross over stimulation of verbal behavior; for example by showing the written form of the word and then asking him to read it aloud or else speak it to the patient for repetition at the same time and by a range of similar language tasks. Conclusion: It is imperative to use combination of constraint and focused upon methods in that direct the patient towards using his or her remaining but unused skills and facilitation of verbal skills through co-relational language processing across different modalities.
Caregiver Burden of Dementia: Correlation with Types and Stages of Dementia and Behavioral and Psychological Symptoms of Dementia
Adreesh Mukherjee, Atanu Biswas, Arijit Roy
Bangur Institute of Neurosciences IPGMER, Kolkata, West Bengal, India
Objective: To evaluate caregiver burden (CB) of dementia and to correlate CB with types and stages of dementia and with behavioral and psychological symptoms of dementia (BPSD). Methods: Study population consists of 60 consecutive patients of dementia and their caregivers. Dementia was diagnosed by DSM-IV criteria. Staging was done by Clinical Dementia Rating (CDR) scale. Standard criteria were used to delineate types of dementia. BPSD were assessed with Neuropsychiatric Inventory (NPI). CB was evaluated with Zarit Burden Interview (BI). Results: Of the 60 patients 34 had Alzheimer Disease (AD) 9 Frontotemporal dementia (FTD) 8 Vascular dementia (VaD) 6 Mixed dementia 3 Dementia with Lewy Bodies (DLB). Average BI scores according to types of dementia- AD 40.5 FTD 55.8 VaD 32 Mixed dementia 47.7 and DLB 46.5. Average BI scores in Global-CDR groups were 11 for CDR-0.5 37.9 for CDR-1 47.8 for CDR-2 and 51.7 for CDR-3. Total NPI scores were: 3.5 for little-or-no burden 13 for mild-to-moderate burden 27.8 for moderate-to-severe burden and 42 for severe burden respectively. CDR- sum of boxes scores were: 3.1 for little-or-no burden 9.3 for mild-to-moderate burden 13.4 for moderate-to-severe burden 13.9 for severe burden respectively. Conclusions: CB varied according to the type of dementia being least in VaD and highest in FTD. CB was more with increasing severity of dementia and increasing BPSD. However moderate-to-severe and severe burden groups had similar severity of dementia. In moderate and severe dementia BPSD seems to have greater effect on caregiver burden than the stage of dementia.
Poster Session 07, Friday, October 02, 2015,
04: 00 PM - 06:00 PM
Dr. Vimla Vermani Hall (Hall D):
An Unusual Presentation of Metabolic Encephalopathy- A Case Report
Rajeswari Daggumati, Sundarachary N V, Veeramma U, Srinivasulu A, Rama Krishna G, Chandana G, Lalitha A, Sridhar A
Guntur Medical College, Guntur, Andhra Pradesh, India
Background: Chronic progressive encephalopathies are a common occurrence in neurological practice. They are a result of several causes like infections metabolic conditions autoimmune and paraneoplastic pathologies. Aim: To present a case of chronic progressive encephalopathy due to metabolic cause. Case report: 32year old gentleman hypertensive alcoholic presented with 4 months duration of progressive neurologic dysfunction characterised by spasticity personality changes cognitive decline pseudobulbar affect all deteriorating into an akinetic mute and rigid status. His laboratory evaluation revealed hyperammonemia hyperargininemia with arginase deficiency which forms the spectrum of urea cycle disorders. Conclusion: Urea cycle disorders are rare but treatable causes of encephalopathy. Hence a high index of suspicion with relevant clinical setting would help establishing the diagnosis of a treatable condition.
Anterior Spinal Artery Syndrome
Madhukar Bharadwaj, SP Gorthi, Vikram Asturkar, Pawan Dhull, YS Sirohi
Army Hospital R&R Delhi, New Delhi, India
Introduction: Spinal cord ischemia accounts for 6% of acute myelopathies and 1-2% of all vascular neurologic pathologies. It presents with different combinations of neurological deficit depending on the vascular territory affected. Its most common risk factors include atherosclerosis and aortic disease followed by aortic dissection. Aims and Objectives: To determine the spectrum and presentation of anterior spinal artery syndrome Settings and Design: This is a prospective observational study conducted at a single tertiary care centre. Materials and Methods: This study included two patients presenting with paraparesis. The patients were diagnosed with anterior spinal artery syndrome based on clinical findings and neuroimaging. Results: The spectrum ranged from brown sequard syndrome to acute onset paraplegia with bilateral spinothalamic loss. Both the patients had developed anterior artery syndrome after bronchial artery embolization. The diagnosis was made based on the presentation and MRI spine which showed the classical owl eye appearance. Both the patients were managed conservatively and showed significant improvement at three months. Conclusion: High index of suspicion is required to diagnose anterior spinal artery syndrome resulting after a procedure which may compromise the spinal cord arterial supply.
A Case Report - Adoloscent Adrenoleukodystrophy Presenting with Raised Intracranial Pressure
Srinivas Raju, B. Nataraju, Sreekanta Swamy, Nischal Reddy, Anantha. G, Sowmya, Gurucharan
Vydehi Institute of Medical Sciences, Bangalore, Karnataka, India
Background: Adrenoleukodystrophy (ALD) is a rare inherited metabolic disease caused by an enzyme deficiency leading to accumulation of saturated very long chain fatty acid(VLCFA)especially in brain adrenal cortex and testis. Case Report: We report a 21 year old presenting with three months history of headache vomiting and progressive vision loss with signs of optic atrophy and CSF protein - 52.8mg/dl. Other neurological examination was normal and MRI features consistent with ALD and an elevated VLCFA confirming ALD. Adoloscent cerebral ALD is a rarity and presenting acutely with raised intracranial pressure as reported in a few childhood cerebral ALD is not documented. To our knowledge this is a first case report from Indian subcontinent of adolescent adrenoleukodystrophy presenting acutely with raised intracranial pressure.
Myriad Presentations of Genetically Proven Mitochondrial Diseases- A Case Series
Anjan Pyal, A K Meena, Rupam Borgohain, S Afsheen Jabeen, Rukmini Mridula
Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
Introduction: Mitochondrial diseases are a group of disorders with complex genotypes and phenotypes. Recent studies revealed numerous pathogenic mitochondrial DNA mutations and mitochondrial deletions. Case history: We describe the clinically diverse multisystem presentation of 3 patients with rare and unique genetic mutations. Our first patient a 26 year old female presented with sub-acute onset of proximal weakness and severe metabolic acidosis with dramatic improvement on administration of thiamine. Her muscle biopsy revealed blue ragged fibers and ragged red fibers. Genetic analysis revealed a pathogenic mutation m.8363G>A in tRNA Lysine gene. The second patient a 11 year old girl with history of failure to thrive since birth chronic recurrent gastrointestinal complaints presented acutely with weakness and sensory impairment. She had a demyelinating sensory motor neuropathy and a myopathic pattern on ENMG. Her MRI brain showed a leukoencephalopathy. Sequence analysis of complete mitochondrial genome homozygous pathogenic mutation c.121C>T (p.R41W) was identified in MPV 17 gene. In our third patient a 31 year old male who presented with speech disturbances limb weakness and gait unsteadiness. On examination he had palatalmyoclonus neuropathy and cerebellar signs. Muscle biopsy revealed a myopathy. Sequence analysis of complete mitochondrial genome showed a POLG mutation. Conclusion: The utility of genetic analysis in the diagnosis of these challenging disorders is vital and necessity for us clinicians to have keen suspicion of these disorders cannot be overemphasized.
A Rare Case of Neurosarcoidosis
Introduction: Sarcoidosis is accompanied by nervous system involvement in 5% of cases which is a rare disease in India. We report this rare case of neurosarcoidosis with sensory system muscle and cranial nerve involvement.
Case Details: A 41 year old male without any premorbid medical illness came with complaints of sensory impairment over right half of trunk deviation of angle of mouth to left side with difficulty in closing right eye blurring of vision of right eye and progressive proximal weakness of both lower limbs of 2 months duration. General examination showed swelling of both parotids with papulonodular rash over skin on trunk and both arms. Fundus examination showed right eye optic disc edema. There was evidence of proximal myopathy from elevated serum creatine phosphokinase level. Fine needle aspiration cytology from both parotid glands was suggestive of bilateral granulomatous parotitis. Skin biopsy was suggestive of sarcoidosis. Magnetic resonance imaging of brain and whole spine was normal. Serum angiotensin converting enzyme level was high. Patient was managed with pulse methylprednisolone followed by maintainence steroids with which he responded well. Patient showed significant improvement in visual symptoms sensory symptoms and proximal weakness. There was resolution of skin lesions. Conclusion: Our case is a rare presentation of sarcoidosis with nervous system involvement in the form of papillitis proximal myopathy and spinothalamic tract involvement. Sarcoidosis has to be differentiated from tuberculosis which is a common entity in India by appropriate investigations. Sarcoidosis has to be considered as one of the differential diagnosis in any patient presenting with multi-axial central nervous system disease.
Mini Midbrain in Midbrain Ischemic Stroke
Deshmukh D S, Muralidhar Reddy Y, Nilesh Vijay Chaudhary, Shyam K Jaiswal, Murthy JMK
The Institute of Neurological Sciences Care Hospital, Hyderabad, Telangana, India
Introduction: Midbrain is supplied by penetrating arteries from posterior cerebral superior cerebellar posterior communicating and anterior choroidal arteries. This report describes hither-to not described sign in two patients with midbrain ischemic stroke. Case1: A 67-yr- male presented with acute onset altered mental status and weakness of all four limbs. Examination revealed a deeply comatose patient with absent dolls eye movement dilated pupils flaccid quadriplegia and bilateral extensor plantar (NIHSS of 18). Magnetic resonance Imaging (MRI) brain showed acute infarcts in bilateral occipitotemporal lobes in addition to mesencephalon. The morphology of acute infarct in midbrain resembled the midbrain in contour. Intracranial MR-angiogram showed non visualization of distal basilar artery and posterior cerebral arteries. Case2: A 43- yr- old male presented with acute onset altered mental status and weakness of left upper limb and lower limb. Examination showed drowsy arousable patient with right complete and left partial third nerve palsy left hemiplegia (NIHSS of 20). MRI brain showed acute infarcts in left occipital lobe and mesencephalon. The morphology of acute infarct in midbrain resembled the midbrain in contour. Intracranial MR-angiogram showed non visualization of distal basilar artery and posterior cerebral arteries. Conclusion: Infarction of bilateral paramedian arterial territory comprising raphe region oculomotor complex medial longitudinal fasciculus red nucleus substantia nigra crus cerebri results in the infarct with contour of midbrain peculiar imaging features on MRI. The contour of infarcted area resembles the contour of midbrain Mini Midbrain in Midbrain. The stroke mechanism was basilar artery atherosclerosis.
A Rare Case of Brain Sagging Syndrome
Anil Kumar Kasukurthy, SV Naveen Prasad, Dhusyanth Babu, T. Naveen, B. Vengamma
Sri Venkateswara Institute of Medical Science, Tirupati, Andhra Pradesh, India
Introduction: Brain sagging syndrome is characterized by low cerebrospinal fluid pressure postural headache and sagging of brain. Salient feature of the syndrome is headache that worsens when the patient is upright and relieved when the patient is recumbent. We report this rare case of brain sagging syndrome following spinal anaesthesia. Case Details: A 30 year old female P2L2 who underwent lower segment Caesarean section under spinal anaesthesia presented one week later with headache seizures and vomiting. No focal neurological deficits noted on examination. Magnetic resonance imaging brain showed bilateral temporo-parietal subdural collection with bilateral centrum semiovale T2 hyperintensities. Brain sagging was evidenced by downward displacement of mamillary bodies and cerebellum below the line drawn tangential to the frontal cranial base. Compensatory engorgement of pituitary gland and indrawing of corpus callosum were noted on magnetic resonance imaging of brain which are features of low intracranial pressure. Venogram of brain was normal. Patient was managed with head down position analgesics adequate hydration and antiepileptics. Patient responded well to treatment. Repeat magnetic resonance imaging of brain done after two months showed complete resolution of imaging abnormalities. Conclusion: We report this case as a rare manifestation of intra cranial hypotension which can have fatal outcome if not identified and treated in time. Our case report highlights consideration of brain sagging syndrome as differential diagnosis in any case of headache with seizures following lumbar puncture.
Clinico radiological profile of patients with low back pain
Srinivasulu Athina, N.V. Sundarachary, U. Veeramma, Sridhar A., Lalitha R.
Guntur Medical College, Guntur, Andhra Pradesh, India
Introduction: Low back pain is experienced any time in a lifetime by upto 80% of the population. It disrupts all the activities of the individual be it social vocational or recreational. Despite its high prevalence low back pain remains poorly understood and inadequately treated. Objectives: To study the clinical and radiological features of patients with low back pain. Materials and Methods: It is a prospective study conducted at Government General Hospital Guntur. Inclusion criteria: All the patients of low back pain admitted to the neurology department. Age 18 to 80 years. Exclusion criteria: Patients who are unwilling to participate. Observations: Total no. of patients(n)=150. 55% were women. Mean age was 47.2. Clinical presentation- central low back pain(88%)neurogenic claudication (45%) nerve root compression (42.6%)and non-radicular referred lower extremity pain (34%). Radiological features-narrowing of the spinal canal(16%) lateral nerve root canals(14.2%)intervertebral neural foramina(26.4%). Conclusion: The incidence of low back pain in our study is highest in the fourth decade. Most cases run a chronic episodic course. Environmental factors influence the onset and course of low back pain.
Hemiplegia-Hemiconvulsion-Hemiatrophy Syndrome (HHH) with Crossed Cerebro Cerebellar Diaschisis (CCCD) - A Case Report
Murarishetty Manjusha, P. Dhairyawan, N. Veena, K. Sateesh, S. Srilatha, P. Soujanya, B. Santosh Kumar, K. Amareshwar Rao, P. Chandrasekhar
Gandhi Medical College, Hyderabad, Telangana, India
Introduction: Diaschisis in neurology implies reduction of function of a part of the brain following interruption at a remote site of an afferent pathway. Each cerebellar hemisphere is intimately connected to the contralateral cerebral cortex through feed-back circuits needed for smooth execution of motor function. The concept of crossed cerebral cerebellar diaschisis following injury (usually vascular) to the motor cortex of one cerebral hemisphere is well described in literature. In our case we highlight MR evidence of structural changes induced in the cerebellum secondary to injury to the contralateral cerebral hemisphere in a patient with childhood onset hemiplegia. Objective: To report a case of HHH syndrome with crossed cerebrocerebellar diaschisis. Materials and Methods: 25 year old male patient presented with left focal seizures with speech arrest staring look and secondary generalization. Patient had history of meningitis with seizures at 5 yrs of age. After five years he developed seizures he developed left hemiplegia which partially recovered with residual deficits. Results: CT brain revealed right cerebral atrophy with ipsilateral dilatation of ventricles on that side. MRI brain: Cerebral atrophy with features of wallerian degeneration of right mid brain. Prominence of cerebellar folia noted on left side suggestive of left cerebellar atrophy consistent with crossed cerebro cerebellar diaschisis with HHH syndrome. Conclusion: Crossed cerebro cerebellar diaschisis is not only a remarkable neurophysiological phenomenon but seems to have a clinical significance. In adult patients with stroke the degree of acute CCCD was shown to be a quantitative indicator of the long-term functional impairment. The age at the time of cerebral injury is an important factor in the pattern of reorganizational changes within the cerebellum.
A Rare Case of Postpartum Hypernatremic Encephalopathy
Kailas Mirche, Mayank Patel, Shalin Shah, Pranav Joshi
NHL Municipal Medical College andVS Hospital, Ahmadabad, Gujarat, India
Encephalopathy in post partum period is a very common condition especially in developing countries like India. Hypernatremia from diverse etiologies presents with varied neurological manifestations dominated by encephalopathy ranging from impairment of cognition to deep coma. We report a rare case of post-partum hypernatremic encephalopathy. 23 year old right handed female postpartum 8th day of third pregnancy admitted with ataxia and dysarthria since 3 days with altered sensorium since 1 day without any history of fever headachevomiting or seizure. She had full term normal vaginal delivery 8 days back. On examination E2M2Vet decerebrate rigidity with pupils bilaterally mid-dilated reacting to light with intact dolls and corneal reflex. DTR were 2+ with mute plantar without neck rigidity. MRI on admission was suggestive of extrapontine myelinolysis. Serum sodium was persistently high >165 and CK total 3826. All other routine investigations were normal including CSF. In view of hypernatremia the above mentioned findings attributed to hypernatremic encephalopathy. Lack of thirst sensation in the presence of dehydration or hypernatremia can occur in central adypsic hypernatremia with hypothalamic dysfunction. Partial ADH secretion defect can be unmasked during pregnancy due to the enhanced peripheral breakdown by vasopressinase. However our patient did not have features of diabetes insipidus and with available data cause of hypernatremia was unclear in our patient. Patient improved with gradual correction of hypernatremia discharged ambulatory after 3 weeks with repeat imaging showing almost complete resolution of MRI changes.
Poster Session 08, Friday, October 02, 2015,
04:00 PM - 06:00 PM
Dr. Vimla Vermani Hall (Hall D): Neuromuscular Disorders
A case of calf muscle hypertrophy in an adolescent female with Pompe's disease.
Suraj Menon M
Govt. TDMCH Alappuzha, Alappuzha, Kerala, India.
Pompe's disease(glycogen storage disease type 2 acid maltase deficiency) is characterised by a progressive myopathy due to accumulation of glycogen in skeletal muscles in addition to various tissues. Late onset Pompe's disease is characterised by slowly progressive proximal myopathy with respiratory muscle involvement. We present the case of a 16year old female presenting with proximal muscle weakness with calf hypertrophy diagnosed to be late onset Pompe's disease by alpha glucosidase enzyme assay. This case highlights the need to consider Pompe's disesase in patients with proximal myopathy even without the presence of overt respiratory muscle involvement and to consider Pompe's disease in the differential diagnosis of calf muscle hypertrophy.
Sleep Profile in Myasthenia Gravis: A Questionnaire and Polysomnography based Study
Rakesh Kumar, M Nagappa, S Sinha, A B TALY, S Rao, C Nayak
NIMHANS, Bangalore, Karnataka, India
Introduction: Disturbed sleep and its impact on Quality of Life (QoL) is under-recognised in myasthenia gravis (MG). Aim: To prospectively assess sleep profile in MG. Methods: Fifty patients on stable drug therapy for at least one month and age and gender matched controls were assessed using standard questionnaires [Epworth Sleepiness Score (ESS) Pittsburgh Sleep Quality Index (PSQI) NIMHANS Comprehensive Sleep Disorders Questionnaire (NCSDQ)]. Twenty five patients and controls each underwent overnight Polysomnography. Result: Myasthenia Gravis Foundation of America (MGFA) grade was I IIA IIB IIIA IIIB and IVA in 11 19 3 10 6 and 1 respectively. Mean PSQI and ESS scores were similar in patients and controls. Patients with abnormal ESS (>10) were older and had greater neck circumference (p: 0.018 &<0.001). Body Mass Index was greater in patients with PSQI > 5 (p: < 0.05). Age gender and clinical severity did not affect PSQI. NCSDQ showed higher frequency of disturbed sleep snoring early morning headache difficulty in initiation and maintenance of sleep in MG. No correlation was found between QOL and ESS or PSQI. Polysomnography revealed that time in bed percentage of wake and N1 latency to N1 and REM and arousal index were increased while sleep efficacy percentage of N2 and REM were reduced in patients. ESS correlated with hypopnea index (r=0.456 p: 0.022) and apnea-hypopnea indices in NREM (r=0.411p: 0.041); PSQI correlated with isolated limb movements (r=0.464 p: 0.019). Conclusion: Patients with even stable MG had abnormal sleep macro-architecture. However theirESS and PSQI were comparable with controls.
Myasthenia Gravis In A Post Coronary Artery Bypass Graft (Post Cabg) - Two Case Reports:
Abdul Malik, Mukesh Kumar, Ahmad Maqsood Dar, Vasundhara Saikia, Khusbu Goel, Joy Dev Mukherji
Background: Among the disorders of neuromuscular junction myasthenia gravis is the most common one. It is a heterogeneous disorder with no specific etiology identified in about 90 percent of cases. However evidence in favour of genetic predisposition to the disorder has been found to exist which is precipitated by the environmental factors 1 There are only few case reports of myasthenia graves occurring in patients after cardiac surgery (post CABG) in the literature so far. Objective: To show the association of myasthenia graves with coronary artery bypass grafting (cardiac surgery). Material and Methods: We are reporting two cases of myasthenia gravis presenting immediately after Coronary artery bypass surgery (CABG). Case 1-70 years old gentleman admitted with triple vessels disease. He underwent CABG successfully. He was extubated as per the post-operative plan but reintubated due to poor breathing response. He improved significantly after intubation. He was again extubated but similar situation continued. Then neurologist opinion was taken. Family members denied any past history of easy fatigability ptosis or bulbar dysfunction. Examination revealed asymmetrical ptosis with bulbar dysfunction with proximal quadriparesis. Possibility of neuromuscular junction dysfunction was kept. Neostigmine test was positive. He was started on intravenous immunoglobulin and later continued on steroid and azathioprine. Serum anti-acetyl choline receptor antibody and anti-MuSK antibody were negative. He recovered completely. Gradually steroid was tapered and switched to azathioprine during OPD followup. Case 2-72 years gentleman admitted with triple vessels disease. He underwent CABG successfully. He was extubated as per the post-operative plan but reintubated due to poor breathing response. He developed sepsis and acute renal failure. Initially it was thought of encephalopathy related to multiple organ dysfunctions. Family members denied any past history of easy fatigability ptosis or bulbar dysfunction. Examination revealed bulbar dysfunction with proximal quadriparesis. Neostigmine test was negative. He was started on intravenous immunoglobulin and later continued on steroid and azathioprine. Serum anti-acetylcholine receptor antibody and anti-MuSK antibody were negative. He was discharged in stable condition with improving bulbar dysfunction. He came again in triage with type 2 respiratory failure after stopping pyridostigmine. He was again advised for intravenous immunoglobulin. He later succumbed to sepsis with multi-organ dysfunction following prolong intubation. Discussion: We report two cases of myasthenia graves in patients few months after cardiac surgery (CABG). Although myesthenia gravis (MG) developing in a patient shortly after the cardiac surgery could be coincidental but as the similar case reports have already been mentioned in the literature it needs to be seriously reported 2. These cases are unique in the way that they presented in immediate postoperative period and both are biochemically negative. Still they improved completely with immunomodulator therapy. These cases remind us about possibility of NMJ disorder or MG in bulbar dysfunction. Conclusion: Although the association between the myasthenia graves developing after the cardiac surgery is rare it should be thought as a possibility in all cases of bulbar dysfunction without any other motor deficit.
Expanding the Phenotype of Facioscapulohumeral Muscular Dystrophy: A rare Association with Becker's naevus: A Case Report
S. Praveen kumar,Janardhan DC, Eshwari, Anita Mahadevan, J. B. Agadi
Bangalore Medical College, Bengaluru, Karnataka, India
The different clinical manifestations of Facioscapulohumeral muscular dystrophy (FSHD) include the well known heterogeneous muscular deficit and the variable presence of any audiological ocular cardiac involvement. Neurocutaneous association with FSHD has not been reported in literature. We report a young boy with the classic clinical picture of FSHD with shoulder girdle and facial muscle weakness of asymmetric onset and he was noticed to be having a hyperpigmentednaevus over his right shoulder and upper arm and associated with mild hypoplasia of right pectoralis major. Dermatology consutation and the literature search typified the naevus to be Becker's naevus which is classified under the Epidermalnaevus syndromes. Histopathological examination of the skin biopsy from the naevus site was confirmatory of this entity. This case merits attention due to the rarity of association of FSHD with Becker's naevus.
Interesting Case Reports of Paraprotenemic Polyneuropathy
R.M. Bhoopathy, R.M. Bhoopathy, E. Uma Maheswari., Yomesh, M. Jawahar
TamilNadu Govt. Multi Super Speciality Hospital, Chennai, Tamil Nadu, India
Introduction: Paraprotenemic poly neuropathy (PPN) is a heterogenous group of neuropathy in which there is excessive amount of class of immunoglobulins (IgG more common followed by IgM & IgA) in the serum. The presence of paraproteins in a case of poly neuropathy may be a chance association or the neuropathy may be secondary to the disease process. Aim: To evaluate the presence of paraproteins in patients presenting with peripheral neuropathy (sub acute onset β€" sensory Motor) and to analyse its significance. Method and materials: We prospectively evaluated 30 patients with sub acute onset β€" sensory Motor poly neuropathy at department of neurology. Out of 30 patients 3 patients were found to have increased paraproteins on electrophoresis. Their clinical profile systemic examination electrodiagnostic assessment serum electrophoresis laboratory evaluation and nerve biopsy were carried out to identify the etiology. Results: Of the three patiens who had paraproteinemia 1 patient was a middle aged female who did not have any systemic features. Electrodiagnostic assessment showed axonal changes and serum electrophoresis showed increased IgG levels and this patient responded well to steroids. The other two patients were elderly males in the 6/7the decade who had systemic features like anemia arthralgia renal failure bone pain lytic bone lesions with a raised IgG/IgM levels. The electrodiagnsotic assessment showed Axonal changes and they were diagnosed to have essential mixed cryoglobulinemia and multiple mylema. They underwent specific therapies for the underlying disease. Conclusion: The presence of paraprotenemia in a case of poly neuropathy may be a chance association or may be causative to the disease process. The strength of the association is assessed based on the clinical neuropathic phenotype electrodiagnostic assessment systemic manifestations and type of paraproteins.
Guillain Barre Syndrome in Pregnancy- a Case series report
P. AsirJulin,Chandramouleeswaran. V, Bhanu. K, Sarala. G, Ramakrishnan
Madras Medical College, Chennai, Tamil Nadu, India
Introduction: Guillain Barre syndrome (GBS) complicating pregnancy is a rare event. GBS in pregnancy carries a high maternal risk and adverse fetal outcome. A high index of suspicion is needed for its diagnosis. GBS should be considered in any pregnant patient complaining of muscle weakness general malaise tingling of the fingers and respiratory difficulty. Objective: We report this case series due its rarity and high index of suspicion needed for its diagnosis. Delayed diagnosis is common in pregnancy because the initial non-specific symptoms may mimic changes in pregnancy. Hence we like to throw light on the need for early diagnosis and effective management. Materials and Methods: Five cases of GBS complicating pregnancy have been analysed. Results and Discussion: Among the five cases 2 cases were Multi gravida and 3 cases were Primigravida. Two of the cases presented during the first trimester one in second trimester and two in third trimester. Antecedent illness was present in 3 cases. The presenting symptoms were same as that of general population in four cases. Atypical presentation was found in one case. Bilateral facial palsy was the presentation in three cases. 3 cases presented as AMSAN and 2 cases presented as AIDP. IVIg was treatment option in all five cases. None of the cases needed ventilatory support. Mode of delivery- two cases labour natural two cases caesarean and one case went in for IUD. All the five cases recovered. Fetal outcome was better. Conclusion: To conclude GBS is rare in pregnancy but can be managed with intensive care involving ventilator support plasma exchange or IVIG and highlights the combined role of gynecologist and neurophysician in the management of GBS during pregnancy which if missed can be detrimental both for mother and fetus.
A Case of Adrenal Adenoma with Myopathy
P. Muthu Kumar, PC. Vijayakumar, K. Venugopal
Sooriya Hospital, Chennai, Tamil Nadu, India
23 years old female complaints of difficulty in using left upper limb one day followed by difficulty in getting up from sitting postureand difficulty in using right upper limb. She had pain in both thighs. No negative sensory symptoms. No bladder bowel disturbances. No bulbar weakness. On examination red macular lesion in left leghigher mental function normal. Fundus normal. Palatal movements normal. Single breath count normal. Muscle bulk normal. She had hypotonia and quadriparesis. All superficial reflexes normalDTR++ Plantar bilateral flexor. Sensory system normal. She had hypokelemiaher serum CPK 2522 her urine spot Na 69 serum magnesium 2.6her thyroid profile normal. EMG suggestive of myopathy. Nerve conduction study normal Muscle biopsy suggestive of myopathy CT abdomen- right adrenal adenoma Serum RA factor positive Plasma renin activity 0.04 ng/ml/hr. ANA positive P-ANCA weak positive she underwent right adrenal adenoma removal After which herquadriparesis completely recovered This patient is reported here for association of adrenal adenoma with rheumatological diseases.
Autosomal Dominant Distal Hereditary Motor Neuropathy Type 2- A Case Report
Institute of Neurosciences SRM Institutes for Med, Chennai, TamilNadu, India
Introduction: Distal hereditary motor neuropathies (dHMN) are genetically inherited slowly progressive length dependent motor neuropathies. We present our patient who was clinically diagnosed as autosomal dominant (AD) dHMN type 2. Case vignette: A 59 year old gentleman presented to our Outpatient services with complaints of longstanding bilateral symmetric weakness of both feet and prominent manifestation as gait instability. Examination revealed bilateral foot drop and absent deep tendon reflexes. He came to our hospital for a specialist opinion since he had been earlier diagnosed as CIDP and was on oral steroids with no improvement in symptomatology. Upper limb nerve conduction studies were normal. Lower limb motor nerve conduction showed nonstimulatable nerves. All the sensory nerve action potentials were normally obtained. Sural nerve biopsy showed evidence of definite axonal degeneration as well as loss of small and large myelinated fibres with sparse inflammatory cells. Detailed history revealed a significant family history with similar illness in his brother father and grandfather. The long duration of neurological illness predominant distal motor weakness autosomal dominant inheritance pattern and absence of sensory involvement made us consider the diagnosis of AD dHMN type 2 in this patient. Genetic confirmation could not be performed. Discussion & Conclusions: 11 subtypes of dHMN have been recognised till date. Out of the six AD subtypes onset at adulthood and predominant lower limb involvement support the diagnosis of AD dHMN type 2. Detailed history focused examination and prudent workup clinch the diagnosis in most cases.
Poster Session 09, Friday, October 02, 2015,
04: 00 PM - 06:00 PM
Dr. Vimla Vermani Hall (Hall D): Infections
Catatonia in Children following Systemic Infections
S.R. Chandra, Thomas Gregor Issac, Sumanth Sivaram
National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
Introduction: The term catatonia was first introduced in 1874 and several etiologies both organic and psychiatric have been attributed to the clinical phenotype of catatonia. The interesting feature is their response to Lorazepam irrespective of their etiology. Patients and Methods: Five patients referred as post encephalitic sequelae but had the unique phenotype with verbal and motor unresponsiveness following febrile illness. They were evaluated for infectivemetabolic and autoimmune causes. Those who qualified for catatonia as per DSM-V criteria and BFCSI screening tool and rating scale were evaluated in detail. Conclusion: Catatonias are not uncommon in children. Usually it occurs in the setting of systemic disease. Nonspecific viral antibodies may be found in the Cerebrospinal fluid. Alteration in Iron binding capacity and ferritin levels can be seen. EEG might show abnormality based on the heralding organic disease. Females are more affected than males and probably occur in clusters suggesting a common underlying etiology in the environment like some unknown neurotropic viruses. Prognosis is good if diagnosed early. Irrespective of the cause patient show good response to lorazepam challenge as well as follow up treatment with lorazepam. None of our patients needed ECT and 75% of our patients became completely normal at one month of follow up. The only patient who had a partial response had duration of illness more than 6 months. Hence it is important to be conscious about the possibility of catatonia in unresponsive children following minor or major systemic disease.
Spontaneous Lower Extremities Swelling Following Albendazole Therapy in a Case in Neurocysticercosis
Abhishek Kumar, Prashant Kumar, Kuljeet Singh Anand, Jyoti Garg
PGIMER (Delhi) &Dr. RML Hospital, Delhi, India
Introduction: Neurocysticercosis (NCC) is caused by larval form of the pork tapeworm T. Solium. Common sites of the cysticerci are brain CSF skeletal muscle subcutaneous tissue or eye. Antiparasitic treatment associated exacerbation of the inflammatory response in brain; eye or subcutaneous tissue is well known. Here we are reporting a case of NCC who developed severe swelling of lower limbs following albendazoletherapy. Case Report: A 40-year-old housewife a known case of multiple NCC and multifocal seizures for 13 years. Her seizures were fairly controlled on antiepileptic medications. She had received antiparasitic treatment in the past. She presented with breakthrough seizures and headache. Her general and systemic examinations including fundus examination were normal. There was no evidence of subcutaneous nodules. MRI brain revealed multiple cysts in varying stages. In view of active disease she was prescribed repeat course of Albendazole along with steroids. On third day of Albendazole therapy she developed swelling and pain of both the thighs and it became more severe in the next one week involving legs also. Doppler study of both the lower limbs was normal. Plain X-ray films showed multiple "cigar-shaped" calcifications in thigh and calf. MRI thigh showed severe muscle swelling in thighs and legs. She was treated with high dose of steroid with subsequent resolution of swelling. Conclusion: Antiparasitic treatment associated inflammatory changes can occur in any tissue having cysticercus. In our case it was in both lower extremity muscles. Therefore one must be vigilant about this rare complication.
Clinical and Radiological Spectrum of Patients with Pott's Spine
E. Uma Maheswari, M. Jawahar, R.M. Bhoopathy
Tamil Nadu Govt. Multisuperspeciality Hospital, Tamil Nadu, India
Introduction: Spinal Tuberculosis (TB) is a common form of Extrapulmonary TB causing significant mobility and mortality in the developing countries. The clinical manifestations are varied ranging musculoskeletal pain to dense neurological deficits. Early diagnosis requires high index of suspicion and early intervention will prevent irreversible neurological damage. Aim: To analyze various clinical and radiological manifestation of Pott's spine. Material and methods: Patients who attended our outpatient / inpatient services and diagnosed as Pottβ spine was registered and analyzed clinically and radiologically. Results: Out of 8 cases with Pott's spine 6 cases were females and 2 were males in the age range of 35-58 years. The presenting clinical features ranged from non specific vague musculoskeletal pain to spastic quadriparisis / paraparesis. The patients who presented with dense neurological deficits gave history of chronic pain in the cervical/lumbar region prior to the onset of deficit. The most common spinal segment involved was dorsolumbar spine followed by cervical spine involvement. The different MRI features observed in these patients were narrowing of disc space involvement of para discal portion of vertebral body complete collapse of body of vertebra with pre vertebral and epidural collections causing compression of cord. Out of 8 patients in three patients we had drained the epidural/paravetebral collections and had subjected them to AFB culture and subjected to tissue diagnosis. They revealed non specific inflammatory changes. Conclusion: Pott's spine must be suspected in people presenting with chronic low back ache. The common age group involved is the middle aged group with a female predilection. Early identification and intervention can prevent the irreversible crippling neurological damage.
Creutzfeldt - Jakob disease -A Case Report
Justin, Ramu, Chezian, Muthukumar, Manivannan, Sritharan, Ganesan
Madurai Medical College Madurai, Madurai, Tamil Nadu, India
Introduction: CreutzfeldteJakob disease (CJD) is a fatal prion-related neurodegenerative disorder. Case Vignette: A 40 year old lady presented to our hospital with Pain and burning sensation involving right side of the body 10 months ago. Four months later she had developed insomnia irritability intolerance to loud sounds and behaviourallteration. She consulted a psychiatrist and was on antipsychotics for a month. Then patient developed slowness of walking with decreased arm swing while walking with a tendency to fall forward. Later she developed Memory disturbancesinappropriate laughter grasping her of both hands dragging of right leg Urinary and fecal incontinence. Later followed by Inability to recognise daughter and husband assaultive behaviour Difficulty in swallowing Inability to walk without support with unsteadiness while walking. I weekprior to admission in our hospital she was not communicating inability to comprehend Not able to walk even with supporttotally bed ridden. She had recurrent episodes of GTCS lasting for 3 minutes. Total duration of illness from the onset to the end is about 10 months duration on Examination she was Unconscious PEARL DEM +localizes painful stimulim rigidity of all four limbs+ DTR brisk plantar-extensor. She had myoclonic jerks involving all the four limbs. No meningeal signs. Fundus- no papilledema. Investigations revealed normal blood sugar RFTLFTelectrolytes. HIV ELISAV TPO antibody were negative. LP and CSF analysis were normal. EEG Bilateral biphasic triphasic wave complex of 1 Hz/sec seen. Bilateral periodic sharp wave complex seen. MRI BRAIN done showed cortical ribbon sign pulvinar sign. Discussion: The early features of CJD vary and are nonspecific. Our patient's clinical presentation EEG findings and MRI Brain provided clues for diagnosis.
A Case of HIV Vacuolar Myelopathy
T.C. Vijay Anand, Dr S. Narendran Sundarapandian, K. Ganesan
Meenakshi Mission Hospital Madurai, Madurai, Tamil Nadu, India
Introduction: Vacuolar myelopathy is a common form of spinal cord disease in HIV positive patients. Case Vignette: A 40 year old female a known case of HIV on ART presented with subacute onset and slowly progressive spastic weakness of both lower limbs. She could not walk without support. Later she developed unsteadinesswhile walking with support and washbasin sign which worsened her to almost bedridden state. Examination revealed normal higher mental function &cranial nerves. Spinomotor system examination showed spastic paraparesis of power 4(MRC grading) with brisk knee jerk and absent ankle jerk with extensor plantar bilaterally. Sensory examination revealed loss of joint position sense and vibration below hip level. Pin prick and temperature sensation were intact. Cerebellum examination normal. No meningeal sign. Other system were normal. Investigations done which showed normal blood sugarRFTLFT and B12 level. Cerebrospinal fluid examination were normal. MRI showed T2 hyperintensity of the posterior columns with no contrast enhancement consistent with vacuolar myelopathy. Discussion: The manifestation of vacuolar myelopathy is variable and often presents as a painless spastic paraparesis that is slowly progressive with a sensory ataxia. Our case is HIV vacuolar myelopathy with peripheral neuropathy. We present this case for the rarity of presentation.
Poster Session 10, Saturday, October 03, 2015,
08: 30 AM - 10:30 AM
Dr. Vimla Vermani Hall (Hall D):
Interesting Presentations of Autoimmune Encephalitis.
Juber D. Shaikh, Sangeeta Ravat, Rushikesh Deshpande.
King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, Maharashtra, India
Introduction: Autoimmune encephalitis is usually due to multiple autoantibodies most common amongst them are anti-VGKC and anti-NMDA receptor antibody. Anti-NMDA receptor encephalitis usually presents with prodromal symptoms followed by movement disorder and autonomic manifestations. Anti-VGKC encephalitis is characterized by subacute cognitive dysfunction seizures hyponatremia REM sleep disorders and neuroradiological evidence of temporal lobe involvement. Both these conditions respond to aggressive immunotherapy. 80% patients show partial or complete recovery. Methods: We describe two cases of autoimmune encephalitis. Case 1: 38 year male presented with 4 month history of slowness of activities of daily living and apathic behaviour. On exam patient had a a mask face frontal lobe release signs hypophonic speech bradykinesia and myoclonus. His anti-TPO antibody was negative. MRI brain showed bitemporal T2/FLAIR hyperintensities without significant contrast enhancement. CSF was normal. Anti-NMDA receptor antibody was positive. Case 2: 54 year old male presented with history of recurrent complex partial seizures forgetfulness especially for recent events. On examination his recent memory was impaired rest exam was normal. His CSF was acellular with raised proteins. His MRI brain showed bilateral amygdala and hippocampal T2/FLAIR hyperintensities with minimal contrast enhancement. His serum anti-VGKC antibody was positive. Both cases were treated with immunotherapy in form of IVIG and IV methylprednisolone. First patient showed significant improvement in gait and second patient is seizure free after treatment. Conclusion: Autoimmune encephalitis should be thought in cases presenting with subacute extrapyramidal symptoms and autonomic features as well as rapidly progressive dementia with seizures especially complex partial seizures. Patients require immunotherapy like IVIG or plasmapheresis with steroids.
A Rare Case of AIDP
S. Saravanan, Radha, Rachael, Bobby
Tirunelveli Medical College Hospital, Tirunelveli, Tamilnadu, India
A 25 year old male patient known type 1 diabetic was admitted in a state of Diabetic Ketoacidosis. He was treated with iv fluids and adequate insulin. He improved well and his blood sugar values normalised. During the period of convalescence he developed rapidly progressive flaccid areflexic quadriparesis without respiratory or bulbar involvement and without sensory or sphincter changes. He was clinically diagnosed to suffer from AIDP which was proved by CSF analysis and Nerve conduction studies. He improved clinically with supportive treatment without residual weakness within 2 months. Acute inflammatory demyelinating polyradiculopathy(AIDP) being precipitated by stressful events like pregnancy surgery etc. are known. This case is presented for rarity of AIDP being precipitated by an episode of DKA.
Autosomal Recessive Form of Occulopharyngeal Muscular Dystrophy in Young: A Rare Entity
J C Maloo, Rishabh Maloo
Background: Oculopharyngeal muscular dystrophy (OPMD) is typically inherited in an autosomal dominant fashion characterized by ptosis difficulty swallowing and late onset proximal muscle weakness. OPMD is rare with a prevalence of 1/100000 worldwide. Case Report: A 25 year old male with the complains of weight loss gradually progressive heaviness of the eyes and inability to lift weights he was earlier able to lift for the past two years. Difficulty in swallowing solids and liquids with coughing; progressively increasing for the past one year without any complaint of painful swallowing or diurnal variation of the complaints. His parents were healthy suffering from no chronic medical illness. He being third of seven sibs with his elder sister and one brother suffering from similar complaints. Examination revealed bilateral non-fatigable ptosis with other cranial nerve functions being intact. Extraocular movements were slightly limited with weakness in bilateral sternocleidomastoid muscles some amount of muscular atrophy was noted in both shoulder girdle and pelvic muscles with normal muscle power and reflexes. His investigations revealed a creatinine kinase of 340 IU. A muscle biopsy could not be performed in limitation to the patients age. Discussion: Scrimgeour(1984) was the first to suggest that a recessive form of oculopharyngeal myopathy with distal myopathy was present in a Melanesian family while Goh et al. (2005) was first to report OPMD in a non-Japanese Asian family. Conclusion: The clinical picture for an Occulopharyngeal Myopathy; autosomal recessive inheritance should also be considered in young patients below the age of 30 years.
Predictors of Outcome in Acute Non-Compressive Myelopathy
Shuchit Pandey, R K Gargdm, H S Malhotradm, R Vermadm, P K Sharmadm, Neeraj Kumardm, K P Malhotramd
King George's Medical University,Lucknow, Uttar Pradesh, India
Introduction: Prospective data is sparse on outcome analysis of acute non-compressive myelopathy. We hereby report the role of clinical imaging and biochemical parameters in predicting the outcome in these cases. Material and methods: Eighty consecutive cases of acute non-compressive myelopathy were enrolled and subjected to clinical MRI and biochemical evaluation. Outcome was labelled as good (modified barthel index mBI>12) and poor (12 including). The predictors of outcome were analysed by SPSS v16.0. Results: Mean age of the patients (n=80) was 29.95(14.12), the ratio of male: female being 3:2. Fifty-seven had good while 23 patients had poor outcome. On univariate analysis whole cord involvement (p=0.027) lower mBI at discharge (p=<0.001) delayed initiation of methylprednisolone (p=0.002) and longitudinally extensive phenotypes (p=0.005) showed significant association with poor outcome. On multivariate analysis delayed initiation of methylprednisolone was the only variable associated with poor outcome. Conclusion: Acute non-compressive myelopathies should be managed promptly as delayed commencement of methylprednisolone is the most important predictor of poor outcome.
An Interesting Case of Lithium Toxicity
P. Sampath Kumar, Sampath Kumar, S Gobinathan, S Sakthi Velayutham, K Malcolm Jeyaraj, P R Sowmini, K S Raja, K. Karunakaran, S. Ravindran, A. Vignesh
Government Stanley Medical College, Chennai, TamilNadu, India
A twenty five year old schizoaffective female on chronic lithium and neuroleptic therapy for 10 years presented with vomiting abdominal pain for 20 days and fever for 2 days tremor in all four limbs urinary incontinence and altered sensorium for a day. On admission she was confused febrile vitals - stable GCS-7/15no meningeal signs fundus -normal cranial nerve examination normal rigidity present in all four limbs all Deep Tendon Reflexes were 2+ and plantar reflex was flexor on both sides. With this clinical background a differential diagnosis of Acute CNS infection neuroleptic malignant syndrome lithium toxicity were considered. On investigation Haemogram serum electrolytes LFT RFT and urine routine were normal. Fever evaluation includingurine and blood culture were negative for infection serum cpk-2388U/L. Patient sensorium worsened(GCS-5/15) on third day with mild renal dysfunction(serum creatinine-2.2mg %). After analyzing the clinical profile with elevated CPK and serum creatinine along with background history of chronic intake of lithium we suspected lithium toxicity and proceeded with serum lithium which was found to be elevated (3.2mmol/L). She was taken for haemodialysis. After dialysis there was a decline in the lithium level and her sensorium improved. Two days after haemodialysis patient had pancerebellar features with mild proximal muscle weakness. On followup two weeks later her cerebellar features and weakness completely improved. So it is concluded that any patient who is on chronic lithium therapy presenting with multiple Neuraxial involvement lithium toxicity has to be suspected. As lithium has a narrow therapeutic window monitoring of serum lithium levels should be done periodically.
Quality of Life in Stroke Survivors Attending a Medical College Hospital in Rural West Bengal
Bitoti Chattopadhyay, Souvik Dubey, Arvind R.V., Vishal Gaikwad, Shankar Nandi, Santi Brata Roy, Debal Laha, A.K. Senapati, S.K. Das
Burdwan Medical College, Burdwan, West Bengal, India
Background: Quality of life is defined based on five dimensions: physical wellbeing material wellbeing social wellbeing emotional wellbeing and development and activity; study of which in stroke survivors is scarce in India. Objective: To determine quality of life in stroke survivors in our hospital patients at presentation6 and 9 months of follow-up. Methodology: Following validated scales were used. Barthel Index (BI) and modified Rankin Scale (MRS) and Short Form 36 (SF-36) for physical function Bengali MMSE and Kolkata Cognitive Screening Battery(KCSB) for cognitive function Geriatric Depression Scale (GDS) and Stroke Impact Scale (SIS) for social function. Results: A total of 65 patients [60% ischemic (IS) 40% hemorrhagic (HGC)] were assessed, put in the table format below MRS(3 or worse score) BI(25 or lower score) SF-36(70 or higher score) KCSB (% with significant cognitive impairment) GDS(% showing significant depression) Isch. Hgic Isch. Hgic Isch. Hgic Isch. Hgic Isch. HgicAt base line 75 84 70 90 90 65 70 30 75 306m F Up 42 60 40 50 50 45 66 22 70 409m F Up 30 22 30 20 40 30 68 22 60 20. Conclusion: Physical quality of life appears more affected in hemorrhagic stroke at presentation and early follow-up whereas cognitive dysfunction and depression are more severe in ischemic stroke. In the second follow up improvement is more apparent in HGC than IS. This is part of ongoing study andwe plan to increase our sample and follow-up periods.
Organophosphosurs Compound Induced Delayed Myelo Neuropathy- A Case Report
Karunakaran K., S. Gobinathan, K Malcolm Jeyaraj, S. Shakthi Velayutham, P.R. Sowmini, K.S. Raja, S. Ravindran, P. Sampath Kumar, A. Vignesh
GovernmentStanley Medical College, Chennai, TamilNadu, India
Introduction: Organophosphate compounds are the organic derivatives of phosphorous containing acids. Delayed motor sensory polyneuropathy has been described in organophosphate compounds Pure motor neuropathy and rapidly evolving delayed myelopathy are extremely uncommon. Here we present a rare case of delayed myeloneuropathy. Case Report: A 28 year old male patient with organophosphate poisoning initially presented with confusional state diarrhea and excessive frothy secretion. After appropriate antidote treatment he recovered completely without any residual neurological symptoms and signs. Two weeks after he started developing severe stiff weakness of both lower limbs which recovered partially over a period of 4 years. On examination patient had proximal and distal weakness of both lower limbs stiffness proximally wasting and flailness distally hyperreflexia of knee jerk and areflexia of ankle jerk with intact sensation. MRI Spine did not show any abnormality. Electrophysiological studies revealed severe axonal neuropathy involving motor neurons and normal SNAPs. Conclusions: Chronic neurotoxicity in OPC poisoning depends on (a) chemical composition of the organophosphate (b) dose systematized (c) the administration of anitcholinergics for cholinergic crisis. The pathology of OPC induced delayed neuropathy involves peripheral distal axonopathy. This manifestation is possibly due to phosphorylation and subsequent ageing of neuropathy target esterase enzyme in peripheral nerves. Inhibition of the same enzyme in spinal cord causes myelopathy. This case is presented for its rarity.
Perils in Missing the Diagnosis: When NM (S & DA) Come Together! A Case report
S Meenakshi Sundaram, Krishnan Swaminathan, Somalinga Nagendran, Suresh Periyathambi, Jude Vinoth, Padmaprakash Gandhirajan
Apollo Speciality Hospital, Madurai, Tamil Nadu, India
Background: Failure to recognize the syndrome of autoimmune encephalitis may result in institution of inappropriate management strategies which may have potentially serious adverse effects. Aim: To report a case of autoimmune encephalitis who developed neuroleptic malignant syndrome requiring dual management strategies. Report of the case: A fifteen-year old girl presented with a three-week history of progressively evolving neurological syndrome characterized by involuntary movements of the left upper limb and face generalized tonic clonic seizures and psychosis. Initial evaluation elsewhere failed to throw light on the diagnosis. Valproate haloperidol and lorazepam were instituted along with psychotherapy. Worsening clinical status and development of rigidity warranted transfer to our tertiary care center. On examination she had perioral dyskinesias recurrent jerks over the face and left upper limb along with generalized rigidity. Serum creatinine kinase level was elevated (5100 iu/L). Neuroleptic malignant syndrome was considered haloperidol was therefore withdrawn. Dantrolene and bromocriptine were administered. A diagnosis of auto-immune limbic encephalitis was pursued. Anti-NMDA receptor antibodies were positive. She responded to immunotherapy with steroid and immunoglobulin with return to premorbid level over eight weeks. Message: High index of suspicion is warranted to diagnose anti-NMDA receptor mediated encephalitis. High potency neuroleptics should be avoided in the management of such patients.
Accidental Non-Fire Carbon Monoxide Intoxication: Clinical Features and MRI Observations
Aruna Ramani K, Nagappa M, Sinha S, Bindu PS, Mathuranath PS, Taly A B
National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India
Background: Carbon monoxide (CO) is reported to be the commonest cause of death by poisoning in the modern world. Accidental non-fire related CO intoxication is less recognized and preventable cause of domestic poisoning. Aims and Objectives: To analyze clinical features brain MRI and outcome in accidental CO poisoning. Patients and Methods: Description of seven patients (M: F 1:1.3) with acute CO intoxication between 2005 and 2014. Results: Mean age at evaluation was 35.86+16.9 years (range: 16 to 53years). Source of CO were gas geyser (n=4) and generator (n=3). Patients presented after mean of 3.24+4.0 months (range: 1day to 1year) following CO exposure. All patients had loss of consciousness during acute intoxication and recovered in 6 to 10 hours. Six patients had impaired cognition one of them manifested six weeks following acute intoxication. Other features included rigidity (n=3) seizures (n=2) dystonia (n=1) dyskinesias (n=1) myoclonic jerks (n=1) generalized tremor (n=1). Brain MRI revealed: T2/FLAIR hyperintensity of pallidum (n=6) cortical gray (n=2) caudate and putamen (n=2) cerebellar white matter (n=1) and dentate hilus (n=1) diffuse leukoencephalopathy (n=3) with restricted pattern of diffusion in two and cerebral and cerebellar atrophy in three and four patients respectively. T1W sequences showed pallidal hyperintensity (n=2) and laminar necrosis (n=2). Six patients were followed up for 14.17+12.9 months (range: 1-30 months). Neurological impairments improved partially but all patients had significant persisting cognitive deficits.
Native Medicine induced peripheral neuropathy
Kalpana R, Muthuraj, Sundar S, Nagaraj V
SRM Medical College Hospital Kattankulathur,Kancheepuram, Tamil Nadu, India
Native medicines intake sometimes produces neurological complications especially peripheral neuropathy. Case report: A middle aged man non alcoholic non diabetic presented with burning feet legs and hands for the past 15 days following intake of native medicine for a low back ache. O/E he was conscious alert clinic ally neurological examination including power ankle jerks joint position sense and vibration were normal. The routine nerve conduction studies of all 4 limbs were normal. Hence a clinical diagnosis of small fiber neuropathy following native medicine intake was made. Work up for the common causes of small fiber neuropathy was negative. The native medicine sample (powder & oil) was sent for toxicology analysis which revealed high sulphur content in it. The LFT & RFT were normal. The patient was given symptomatic treatment for neuropathic complaints. The patient is under follow up for the past 4 months and is symptomatically better. In our patient the possibility of sulphur inducing small fiber neuropathy was considered. Conclusion: This case is presented to highlight that sulphur in as native medicine can cause small fiber peripheral neuropathy.
Poster Session 11, Saturday, October 03, 2015,
08: 30 AM - 10:30 AM
Dr. Vimla Vermani Hall (Hall D): Stroke
CT angiogram as a predictor of outcome in symptomatic internal carotid artery occlusion
Soumya Sundaram, Sylaja PN, Santhosh Kannoth, Bejoy Thomas
SCTIMST, Thiruvananthapuram, Kerala
Background: Several studies have showed that in symptomatic internal carotid artery occlusion the extent of infarct stroke severity and outcome depends on the effectiveness of collateral circulation. Aim: To study if collateral circulation as assessed by CT angiogram can predict the 90 day outcome in patients with symptomatic internal carotid artery (ICA) occlusion. Methods: Sixty-five consecutive patients with symptomatic ICA occlusion (January 2011 to December 2013) were included in the study. The collateral vessels (ophthalmic artery anterior and posterior communicating artery and the leptomeningeal vessels) were assessed by CT angiogram. The outcome at 90 days was defined as excellent if modified rankin scale (mRS) βʹ 2. Results: The mean age of subjects was 57 Β11.6 (range 32-80) and 92% were males. Thirty one (47.7%) patients had good outcome (mRSβʹ2). The etiology of ICA occlusion (atherosclerotic vs dissection) was predictive of good leptomeningeal collaterals (72% vs 28% respectively; p=0.03). The excellent outcome was associated with the presence of good leptomeningeal collateral circulation (90.3% vs 9.7%; p <0.05). Among 35 patients who had no collaterals or 1 collateral vessel 22 (62.9%) patients had poor outcome while 13 patients (40%) with 2 or more collaterals had poor outcome (p=0.07). In multivariate analysis only absence of ipsilateral ophthalmic artery (OR 6.4; CI 1.34-30.42) and poor leptomeningeal collateral circulation (OR 4.85; CI 0.96-24.55) were independent predictors of poor 90 day outcome. Conclusion: Assessment of collateral circulation using CT angiogram can help in predicting the outcome of patients with symptomatic ICA occlusion.
Early Recurrent Ischemic Stroke: Risk Factors Estimation-A Prospective Study from Tertiary Care Teaching Hospital Of Western Rajasthan
MD Yasin, Md. Yasin, A. Bhargava, K. Shubhakaran, Janardan, Ankit Shaha, K. Sachdev
Dr. S. N. Medical College, Jodhpur, Rajasthan, India
Background: Prospective data are lacking for risk factors of early recurrent stroke in Indian context. Aim: Study to identify clinical predictors of early recurrence after ischemic stroke. Methods: Prospective observational study (September 2013 to April 2015) Of 297 patients (Male 196 Female 101 Mean age 62Β8.4) of ischemic stroke to identify recurrent stroke occurring within 90 days of index stroke. Survival free of recurrence was estimated using Kaplan Meier analysis stratified by demographic variables vascular risk factors discontinuation of antiplatelet stroke syndrome subtype vascular territory; score on Barthal Index(BI) and MMSE during hospitalization. Results: We identified 22 recurrent event in first 90 days after index stroke resulting in a recurrence rate of 7.4%. Among demographic characteristics we found no effect of age sex education. There was a trend towards higher rate of early recurrence in those who discontinued antiplatelet; 10 recurrence in 82 defaulters (12.2%) Vs 11 in 215 Antiplatelet adherent patient (5.11%) which was significant (p<0.001). Among vascular risk factors a higher risk of recurrence among patients with hypertention 7.92% Vs Nonhypertensive 6.10% Diabetic 6.21% Vs nondiabetic 4.94% Smoker 8.64% Vs Nonsmoker 5.44% but those difference failed to reach statistical significance. Conclusion: Although vascular risk factors like hypertension diabetes smoking alcohol are well known risk factors for recurrent stroke. In our country discontinuation of antiplatlet (aspirin or clopidogrel) is also a major culprit. Efforts to sensitize patients for adherence to antiplatelet must be carried out.
Analysis of Clinical Symptoms & Etiological Factors in Cerebral Venous Sinus Thrombosis Patients: A Retrospective Study
M. Jawahar, R.M. Bhoopathy, Uma Maheswari, Youmesh
Omandurar Multisuperspeciality Hospital, Chennai, Tamil Nadu, India
Introduction: With advent of MRI Neuroimaging the frequency of identifying Cerebral Venous Thrombosis (CVT) have increased. Even though CVT is more common in females incidence of CVT in males is increasing due to increased consumption of alcohol. Aim: To analyze various typical & atypical clinical manifestations of CVT and their different etiological factors. Materials and Methods: 14 patients (Male = 9 Female = 5) with CVT in the age group of 20 β€" 53 years admitted in our Govt. Multi super speciality hospital Chennai over a period of 6 months were evaluated. All 9 male patients were chronic alcoholics. All patients underwent MRI Brain & Magnetic Resonance Venogram (MRV) followed by screening of all hypercoagulable disorders. Their alcohol consumption hormonal pill intake and smoking habits were also analyzed. Results: Clinical symptoms in 14 patients with CVT showed holocranial headache &vomiting (n =14) seizures (n=6) diplopia (n=8) altered sensorium (n=2) Right arm monoparesis (n=1) Left homonymous hemianopia (n=1) and Right optic atrophy (n=1). Signs of papilloedema were observed in 9 patients. Superior saggital sinus thrombosis (n=9) and sigmoid & transverse sinus thrombosis (n=5) were observed in MRI brain with MRV imaging. Etiological factors identified were polycythemic in 3 patients hyperhomocysteinemia (alcohol induced) in 2 patients hyperfibrinogenemia in one patient 2 females were APLAS positive and one female was a migraineur who took OCP. Discussion: Chronic alcoholism causes CVT due to dehydration and by inhibition of antidiuretic hormone which results in extra vascular volume reduction and thereby increased PCV. Liver cell dysfunction due to chronic alcoholism causes secondary increased erythropoiesis and thereby increased PCV. Conclusion: In our part of the country the increasing incidence of CVT in male population is due to the increased consumption of alcohol and its side effects.
Ophthalmic Artery Occlusion With Ophthalmoparesis in Spontaneous Carotid Artery Dissection: An Unusual Occurence
Kiran Buddaraju, Rohan Mahale, Anish Mehta, Abhinandan Ks, Aju Abraham John, Madhusudhan Bk, Suryanarayana Sharma, Mahendra Jv, R Srinivasa
MS Ramaiah Medical College, Bangalore, Karnataka, India
Spontaneous carotid arterial dissection is a nontraumatictear or disruption in the wall of the internal carotid artery(ICA) without a clear etiology. It represents a major causeof stroke in younger patients comprising about 10β€"25%of ischemic cerebral events. Patients can present with arange of symptoms from being completely asymptomaticto having facial/neck pain headaches Hornerβ€™s syndromeamaurosis fugax or transient ischemic attacks (TIAs) andstroke. Isolated ophthalmic artery occlusion (OAO) asa presenting manifestation of spontaneous carotid arterydissection is rare. Here we report a middle-aged male patientwho presented with acute spontaneous right carotid arterydissection with monocular visual loss of the right eye dueto OAO.
Bilateral Sudden Visual Loss Due To Isolated Symmetrical Lateral Geniculate Body Hemorrhagic Lesions.
Raghunandhan Nadig, Delon D'Souza, Thomas Mathew, GRK Sarma, Arvind Prabhu
St John', Bengaluru, Karnataka, India
Introduction: Acute bilateral visual loss is usually caused by post geniculate optic pathway lesions. A rare cause can be bilateral isolated lesions of the lateral geniculate bodies (LGB). Objectives: To describe a rare case of bilateral LGB hemorrhagic lesion causing sudden visual loss and to review similar cases reported in literature. Materials and Methods: An index case of sudden bilateral visual loss due to isolated bilateral LGB lesion was evaluated thoroughly. We did a pubmed and medline search for similar cases. We analysed the clinical scenario and etiology in all the patients who had this rare condition. Results: Index case was a 42 year old lady who presented with sudden visual loss of 4 days duration. The patient had a short febrile illness with hepatorenal dysfunction of probable infectious etiology. MRI showed features of bilateral hemorrhagic infarctions in LGB. Seven cases have been reported in literature from 1933 to 2007. Interestingly all cases have been reported in females with their age ranging from 18 to 37 years. In four patients a possible microangiopathy or hypoperfusion was considered to be the cause. Two of them had pancreatitis one had anaphylactic shock and one had preeclampsia. Associated infections were seen in two other patients including syphilis and febrile gastroenteritis. One patient had hyponatremia and a possible osmotic demyelination. Conclusion: LGB hemorrhagic lesions are an extremely rare cause of acute bilateral painless visual loss and can result from various systemic and metabolic disorders. As of now these lesions are reported only in females.
A Study of Risk Factors And Outcome of Recurrent CVA
Ravi Kiran Padala, N.S. Sampath Kumar, M. Rajaguru
Nellore Sripotti Sriramulu Nellore District, Andhra Pradesh, India
Introduction: Recurrent stroke is a new cerebrovascular event (CVA) which occurs after stabilization of previous stroke. Recurrence of stroke increases morbidity-mortality. Objective: To study relationship between risk factors and outcome of recurrent stroke. Methods: This short term prospective study was conducted in Narayana Medical College in 50 recurrent stroke patients. Previous stroke history was collected from history and old imaging data. Risk factors noted were hypertension (HTN) dyslipidemia diabetes mellitus atrial fibrillation coronary artery disease smoking and alcohol and poor drug compliance. Outcome was evaluated by NIHSS and mRS scores during admission discharge and at 3 months. Results: Among 50 patients 36 male and 14 female; mean age of 59.66 10.269. 47 had ischemic stroke3 had hemorrhagic stroke in previous CVA whereas in recurrent stroke42 had ischemic stroke8 had hemorrhagic stroke. Common risk factors being HTN(n=42) and dyslipidemia(n=40)10 had poor drug compliance history.47 had more than single risk factor and 3 had only one risk factor. Time period between first episode of CVA and recurrence range from 3 months to 8 years. On follow-up 46 are alive have better mRS & NIHSS scores2 died with complications; 2 were lost to follow up. A significant value (<0.001) seen between type of stroke presentation in previous and recurrent CVA. Conclusion: Our study data suggests stroke recurrence is mostly multifactorial; HTN and dyslipidemia being more important risk factors. Stroke outcome is directly proportional to site and extent of brain involved rather than recurrence.
Alein Limb Phenomenon in Pontine Hemorrhage- A Rare Presentation
Mahendra Javali, Aju Abraham John, Masoon Mirza Abbas, Rohan Mahale, Anish Mehta, Abhinandan K.S., Kiran Buddharaju, Srinivasa R
M.S. Ramaiah Medical College, Bangalore, Karnataka, India
Background: Alien limb phenomenon is an interesting motor feature characterized by an unwilled and uncontrollable but seemingly purposeful and goal oriented movements of an upper limb where the hand moves as if it had a mind of its own. A frontal callosal and posterior type has been described with typical clinical features. Objectives: To study the clinical features and localization of deficits in a middle aged gentleman presenting with right pontine hemorrhage and a left alien limb phenomenon. Methods: Review of literature showed that frontal or callosal type are more common but parietal cortex or thalamus lesions causing alien limb phenomenon have also been described often. Subcortical structures producing alien limb is rare. We evaluated for probable explanations and other possibilites to the patientβ€™s presenting symptoms. Results: The patient had transient features suggestive of an alien limb phenomenon in his left upper limb which resolved with prior residual deficits of pontine hemorrhage. Conclusions: A lesion from a subcortical structure such as pons causing an alien limb phenomenon is distinctly rare and the pathogenesis may be related to changes in body schema associated with a lack of sensory feedback as seen in the posterior type of alien limb phenomenon.
Poster Session 12, Saturday, October 03, 2015,
11: 00 AM - 01:00 PM
Dr. Vimla Vermani Hall (Hall D): Epilepsy
A Rare Case of Non Ketotic Hyperglycinemia Presenting as Neonatal Refractory Seizures
Leema Pauline C, Viveka Saravanan, Ravi
Institute Of Child Health &Hospital for Children, Chennai, Tamil Nadu, India
Case Vignette: A 12 day old term newborn first born of third degree consanguineous marriage was referred to our hospital as refractory seizures. The baby had focal clonic seizures from first day of life multiple episodes per day. Antenatal period was uneventful. The child was born of normal vaginal delivery with a birth weight of 2.7 kg with no evidence of birth asphyxia. There was no history of feeding difficulty or abnormal urine odour. There was no family history of seizures either. On examination the child was lethargic. There was no dysmorphism or neurocutaneous markers. Investigations: Blood sugar ionised calcium and magnesium levels were within normal limits. USG cranium was normal; sepsis screening was negative; CSF analysis including sugar was normal; empirical pyridoxine biotin trial could not achieve seizure control. There was no metabolic acidosis and urine ketones were negative. Urine metabolic screening was negative. Serum lactate ammonia were normal. Tandem mass spectrometry and serum biotinidase level were normal. MRI brain including MRS were normal. CSF glycine was elevated (184 ng) and CSF/serum glycine ratio (0.45) was also elevated. Conclusion: A diagnosis of non-ketotic hyperglycinemia was established. Seizures were controlled with three antiepileptic drugs and sodium benzoate. This case is presented for its rarity.
Clinical Profile of Patients With Psychogenic Nonepileptic Seizures And The Utility of Suggestion Techniques
Prasanth S R,Shaji C V, Kabeer A, Ram Mohan, Parvathy G, Suraj Menon, Meenakumari
Background: Psychogenic nonepileptic seizures (PNES) are paroxysmal events that are often misdiagnosed as epilepsy and leads to the unnecessary use of antiepileptic drugs. The clinical profile of 56 patients with PNES and the usefulness of suggestion techniques in same is reported here. Methods: All patients presenting with clinically suspected PNESaged 12 years or moreduring the period September 2013-March 2015 were included. Those with coexisting epilepsy were excluded. Data including age and gender distributionsemiology of episodes and any comorbid psychiatric illness were collected. Detailed neurological examination and mental status examination was done. All underwent 19 channel digital video electroencephalography (EEG) of 40 minutes duration. Out of 56 every alternate patient (n=28) was allotted to suggestion groupwho were given simple suggestions that seizure may occur during recording. Any abnormal motor activity if found were reinforced by further suggestions. The other group (n=28) was not given any suggestions. Results: Out of 56 patients 44(78.5%) were females34 (60.7%) were in the age group 12-20yrs.24 patients (42.8%) were already receiving antiepileptic drugs. Main psychiatric comorbidities were depressive disorder in 48 (85.7%)anxiety disorder in 37(66%) and personality disorders in 21(37.5%). In the suggestion group19 of the 28 patients(67.8%) had events that were identified as non-epileptic seizures(without any EEG abnormality) and confirmed as habitual attack by eye witnesses. Only 6 of the 28 patients(21.4%) in the no-suggestion group had habitual attacks. This was statistically significant difference (p value 0.001) with an odds ratio of 7.714. Conclusions: A short outpatient video EEG record is often sufficient to diagnose PNESif supplemented with proper suggestion techniqueswhich would otherwise require prolonged inpatient video EEG recording.
Clobazam Induced Pedal Edema: Unrecognised Side Effect
Delon D'Souza,Thomas Mathew, Raghunandhan Nadig, GRK Sarma
St. John's Medical College, Bengaluru, Karnatka,India
Introduction: Clobazam is considered a relatively safe anti epileptic drug with fewer side effects like sedation. Here we report four cases of epilepsy who developed pedal edema after starting clobazam. Objectives: To study the occurrence of clobazam induced pedal edema in epileptic patients Materials and Methods: Patients of epilepsy who developed pedal odema after starting clobazam were evaluated in detail. Temporal relationship between starting clobazam and appearance of pedal edema was ascertained. Other causes of pedal edema were excluded by appropriate biochemical tests and sonological evaluation. All patients were followed up after stopping clobazam to confirm the complete resolution of edema. Results: From July 2014 to May 2015 we had four epileptic patients who complained of pedal edema after starting clobazam. Three were females and one was a male patient. Their ages ranged from 18 to 58 years. All 4 patients had complex partial seizures and clobazam was introduced as add on therapy. All 4 patients developed pedal edema within 3 to 4 weeks of starting clobazam. Patients had pitting non painful edema with one of the patients developing anasarca. Evaluation for other causes of pedal edema was negative. One patient was on valproate which is known to cause leg swelling. None of the other patients were on any drugs that are known to cause pedal edema. Pedal edema completely resolved once clobazam was stopped. Conclusion: Pedal edema appears to be one the side effects of clobazam treatment. Physicians and neurologists should be aware of this rare side effect of clobazam to avoid unnecessary and elaborate investigations in these patients.
An Algorithm for Detection of Epileptiform Discharges
Atri Chatterjee, Khakon Das, Pramit Kr. Ghosh, Shankar Prasad Saha
Nil Ratan Sircar Medical College, Kolkata, West Bengal, India
Epileptiform discharges are characterized by an abrupt disruption of the background rhythm of the electroencephalogram (EEG). However this change is visible to the naked eye only if it crosses a certain threshold. We developed an algorithm based on the basic properties of epileptiform discharges to analyze EEG segments that do not show any obvious discharge. The algorithm was tested in a single-blind manner on EEG from both epileptic patients and normal controls. A total of 150 EEGs were analyzed and compared with clinical data and visual EEG interpretation. The algorithm had a sensitivity of 91% and a specificity of 97.4% in discriminating abnormal from normal EEGs. We propose that this algorithm can supplement the EEG reader in discovering epileptic form discharges specially in situations of long-term monitoring and high density electrode arrays.
Predictive Factors of Seizures in Cerebral Venous Sinus Thrombosis
Rohan R Mahale,Anish Mehta, Aju Abraham John, Kiran Buddaraju, Abhinandan KS, Madhusudhan BK, Suryanarayana Sharma, Mahendra Javali, R Srinivasa
MS Ramaiah Medical College, Bangalore, Karnataka,India
Background: Seizures are the presenting feature of cerebral venous sinus thrombosis (CVST) in 12 31.9% of patients. 44.3% of patients have seizures in the early stage of the disease. Seizures can occur during different periods of the disease course. Acute seizures (AS) refers to seizures which take place before the diagnosis or during the first 2 weeks afterward and remote seizures (RS) which occur in the years following CVST development. Mortality in patients with seizures has been reported three times higher than in those without seizures. Objective: To report the predictors of acute seizures in cerebral venous sinus thrombosis (CVST). Methods: 100 patients with CVST were included in the study. The occurrence of acute seizures was noted. The baseline characteristics of the patients with and without acute seizures were compared. The predictors of acute seizure were evaluated by univariate analysis including the demographic (gender age) clinical (headache focal neurological deficit papilloedema GCS score) type and number of risk factors MRI findings (Type of lesion: haemorrhagic infarction or hematoma location of lesion) and MRV findings (superficial or deep sinus cortical veins). Results: A total of 48 patients with CVST had acute seizures (focal - 6 focal with secondary generalized - 18 and generalized tonic clonic -24) of whom 7 had status epilepticus. On univariate analysis temporal (P = 0.01) and parietal lobe (P = 0.02) involvement haemorrhagic infarct (P = 0.002) and transverse/sigmoid sinus involvement (P= 0.01) were associated with higher risk of acute seizure. Conclusion: Transverse/sigmoid sinus involvement temporal and parietal lobe involvement and haemorrhagic infarct were associated with higher risk of acute seizure in patients with CVST.
Role of EEG in Predicting Seizure Relapse During or After Anti-Epileptic Drugs Withdrawal
Biplab Das, Parampreet Singh Kharbanda, Manoj Kumar Goyal, Vivek Lal, Sudesh Prabhakar
Post Graduate Institute of Medical Education and Research, Chandigarh,India
Background: The role of electroencephalography (EEG) in predicting seizure recurrence during or after antiepileptic drugs (AED) tapering is debateable. Objective: To study the role of EEG in predicting seizure relapse during or after AED withdrawal who were seizures free for at least 2 years duration and to look for any correlation between seizure free duration and relapse. Materials and Methods: In this prospective study 100 epilepsy patients (56% idiopathic 44% symptomatic) with minimum 2 years seizure free were followed-up for atleast 6 months after AED tapered off. EEG abnormalities were classified as using PGI modification of Mayo classification system. Results: The overall risk of seizure recurrence was 42% (24 patients had recurrence during AED tapering 13 patients had within 3 months while 5 had recurrence after 3 months of AED tapering). Past history of failed AED tapering and prolonged active seizure had high risk of seizure recurrence (p=0.001 0.003 respectively). EEG abnormalities (Grade 2) in baseline or follow-up were associated with significantly higher risk of recurrence (p=0.0001). All three types of EEG abnormalities (dysrhythmia asymmetry and delta activity) were correlated significantly with seizure recurrence although it was highest with grade 3 dysrhythmia (p<0.001 RR=25.18 PPV=81.25%). But types of seizures seizure semiology or seizure free duration had no significant bearing on seizure recurrence. Conclusion: Abnormal EEG recording (grade 2 or dysrhythmia) can predict seizure recurrence. Prolonged or serial EEG recording significantly increases the yield of picking up abnormalities. Seizure free durationdid not predict relapse rate although seizure active duration was important.
Knowledge Attitude and Practices of Doctors on Pregnant Women with Epilepsy (KAP Study)
Lalitha Rajanala, N.V. Sundarachary, U. Veeramma, Chandana G, Srinivasulu A
Guntur Medical College, Guntur, Andhra Pradesh,India
Background: Pregnant women with epilepsy constitute a special cohort. There are many medical issues that require both obstetric and neurological expertise. In our country most of these patients are under the care of general physicians or obstetricians. Aims and Objectives: To conduct a KAP study among doctors about issues in managing pregnant women with epilepsy. Methodology: A total number of 150 doctors (50 each of physicians obstetricians and neurologists) with minimum of 3 years of experience were asked to fill up a validated KAP questionnaire and the data was analysed. Results: Of the 150 doctors studied 96 were from urban areas and 54 were from rural areas which did not show difference in the knowledge about evidence based management but showed a difference in Antiepileptic drug selection. Positive attitude was observed with respect to adapting to newer guidelines and innovative strategies. Conclusion: Our study showed significant differences in knowledge attitude and practices of doctors in rural and urban areas across specialities in the approach and management of pregnant women with epilepsy.
Case Series of Recurrent Seizures in Children
R. Rajarajan, C. Leema Pauline, V. Chandramouleeswaran, V. Ramakrishnan, R. Vivekasaravanan, K. Bhanu
Madras Medical College, Chennai, Tamil Nadu,India
Introduction: Many patients with newly diagnosed epilepsy have multiple episodes of seizures before diagnosis. Often recurrent seizures are necessary to establish a diagnosis. Patients with seizures due to metabolic derangements are generally not felt to be at risk for future epilepsy but they are prone for seizure recurrence in acute setting. Results: 5children presented with recurrent seizures which was not controlled with adequate & appropriate antiepileptic drugs. On evaluation these children were found to have seizures secondary to metabolic derangements. 2cases were found to have hypoglycaemia secondary to addisons disease and fatty acid oxidation defects. 2cases had hypocalcemia secondary to vitamin D deficiency & primary hypoparathyroidism. 1case had hypoglycaemia on multiple occasions in which the cause of hypoglycemia could not be identified. Once the metabolic parameters were normalised seizures were controlled and short course of AEDs were tapered & stopped. Conclusion: A high index of suspicion is required to investigate for metabolic causes in all children with recurrent seizures.
A Study of Factors Contributing to Quality of Life in Epilepsy
M. Sivaji, Praveen Hariharan, Thamizhpavai, R. Lakshminarasimhan, K. Banu
Institute of Neurology Madras Medical College, Chennai, Tamil Nadu, India
Aim: To study the influence of factors such as gender socioeconomic status duration frequency of seizures and monotherapy/polytherapy on quality of life in epileptic patients. Methods: Data were collected from 30 patients with more than 1 year of follow up in epilepsy clinic were interviewed with the standard QOLIE 31 questionnaire and patients epileptic history was recorded. Patients with other physical and psychiatric illness were excluded. Results: In this study 40% males; 60% females with epilepsy of which 70 % had GTCS and 30 % had partial seizures. QOLIE T score ranged from 30 to 68 (Mean 49.9910.55). The mean overall T score among males was 47.5 11.8; among females was 51.66 10.14. The mean overall T score for patients with polytherapy was 43.25 11.615 while mean overall T score for patients on treatment with phenytoin and carbamazepine were 49.75 9.65 and 53.66 8.96 respectively. Duration of seizures significantly impaired the quality of life in epileptic patients. The mean overall T score for patients with duration of seizures less than 7 years was 54.33 9.52 and in patients with duration more than 7 years was 43.71 8.88 (P=0.0281). Frequency and type of seizures does not significantly impair the quality of life in these patients. Total scores for each item in QOLIE 31 were also analysed. Conclusion: Duration of seizures significantly impairs the subjectively perceived quality of life in patients with epilepsy.
Poster Session 13, Saturday, October 03, 2015,
11: 00 AM - 01:00 PM
Dr. Vimla Vermani Hall (Hall D):
A Case Report on Kearns-Sayre Syndrome Variant
Jasminekalyani P,Thomas Edwin Raj
Tirunelveli Medical College, Tirunelveli, Tamil Nadu, India
Kearns-Sayre syndrome is a rare disorder. Prevalence of mitochondrial deletions is 1.6 cases per 100000 populations. Our case fulfills the criteria for Kearns-Sayre syndrome without cardiac conduction defects. According to literature Ophthalmoplegia plus syndrome (Kearns Sayre Plus syndrome) is a multisystemic disorder with severe muscle mitochondrial abnormalities. Prevalence of ophthalmoplegia plus syndrome is unknown. We present this case because of its rarity.
Calf Hypertrophy in Myotonic Dystrophy Type1- A Novel Finding
Vinit Banga, Suman Kushwaha
Institute of Human Behavior and Allied Sciences, New Delhi, Delhi, India
Objectives: Calf hypertrophy in Myotonic Dystrophy type1-A novel finding. Background: MD-1 is one of the common muscular dystrophies which clinically presents as progressive myopathy myotonia and multi-system involvement. It is characterised by AD inheritance pattern with presentation from congenital to adult onset. Calf hypertrophy has not been described in MD-1. Methods: Detailed clinical history neurological examination EMG neuro-imaging and genetic testing. Two of our patients both young males with positive family history presented with symptoms suggestive of myotonia weakness frontal baldness along with calf hypertrophy on examination. Results: Our first patient a 26/M presented with complaints of stiffness in both hands of 8 years duration with history suggestive of myotonia facial and generalised weakness (proximal>distal) frontal balding along with history of delayed developmental milestones poor scholastic performance ad decreased cognition. There was positive family history with father and twin sibling having similar illness. The second patient 35/M with normal development & cognition presented with a similar history as above for 2.5 years. His father along with all his siblings were having similar illness. On examination weakness was confirmed and both were found to be having calf hypertrophy. Percussion myotonia was elicited which was confirmed on EMG and a diagnosis of childhood onset MD-1 was made which was confirmed by genetic testing. Conclusion: MD-1 is a multisystem disease with AD inheritance due to CTG repeat expansion in DMPK gene on chromosome 19. It usually presents as progressive weakness(predominantly distal followed later by proximal) myotonia and mulitsystemic features. The atypical features in our case were predominantly proximal weakness which is more commonly found in MD-2 and presence of calf hypertrophy which has not been described in MD-1.
Clot Length and Outcome of Intravenous Thrombolysis in Cardioembolic Strokes
Shruthi Kamath, Dheeraj Khurana, Vivek Lal, N Khandelwal, Chirag Kamal Ahuja
PGIMER, Chandigarh, Punjab, India
Introduction: Successful recanalization of large vessel ischemic stroke after IV-tPA (intravenous tissue plasminogen activator) depends on several factors. Clot length has been shown to predict successful recanalisation and thus outcome after IV-tPA in middle cerebral artery stroke. Studies till now did not determine difference in clot length in cardioembolic and non-cardioembolic strokes for predicting recanalisation. Thus we aim to determine if clot length cut off for successful recanalization in anterior circulation stroke is different for cardioembolic and non-cardioembolic strokes. Materials and Methods: Hospital based prospective observational single blind study. Patients presenting to PGIMER between July 2014 and June 2015 with acute ischemic stroke who were thrombolysed were included. The patients were categorized into cardioembolic and non-cardioembolic stroke and followed up for 90 days post-thrombolysis. Inclusion criteria: Acute ischemic stroke receiving IV rTPA within 4.5 hours with CT angiography showing complete occlusion of ICA tandem ICA+MCA isolated M1 or M1+M2 segments of middle cerebral artery (MCA) and had follow-up CTA between 244 hours of thrombolysis with 90 days follow-upExclusion criteria: Hemorrhagic stroke received only endovascular therapy. Results: 13 patients were included from about 50 patients. Among these 7 received only IV rTPA and others received bridging therapy. 8 patients had cardioembolic stroke. Successful recanalisation with IVT alone (TICI score of 2b and 3) was seen in 23.07% (n=3). Good clinical outcome (mRS οͳ 2) was seen in 38.47% of patients. Relation between clot length and stroke sub-type will be presented later in the platform.
Calcification here calcification there calcification everywhere…A case of multiorgan dystrophic calcification presenting as seizures
Irfan Yousuf Wani, Sawan Verma, Ravouf Asimi, Maqbool Wani, Ravouf Asimi, Maqbool Wani, Irfan Shah, Mudasir Shah, Nawaz Sheikh, Atif Kawoosa
SKIMS, Srinagar, Jammu & Kashmir, India
Calcium is a mineral which is essential for life. A abnormal change in the serum level of calcium in humans can be detrimental. Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone but calcium can be deposited abnormally in soft tissue. Depending upon Calcium metabolism it is of two types Dystrophic calcification and Metastatic calcification. We present a young female who was admitted with generalised tonic clonic convulsions and on evaluation was found to have calcification in brain lungs lymph nodes kidneys and spleen. Her metabolic and hormonal profile was normal. Despite extensive evaluation the cause of multiorgan calcification could not be found. She was managed symptomatically with antiepileptics drugs and is symptom free on follow-up.
Clinical Profile and Outcomes of Unknown Patients Presenting To Neurology Casualty at a Tertiary Care Centre In South India
Ravi Yadav, Umesh, Naveen Kumar C, Guru S, Dwarakanath Srinivas, Rose Dawn Bharath, Suresh Bada Math
National Institute of Mental Health and Neuroscien, Bangalore, Karnataka, India
Objectives: Unknown (without any details) patients pose a unique problem in the neurological emergency services of India in context of legal issues humanity and treatment issues. These patients pose a diagnostic and management challenge to treating physicians and staff. The primary objective of the study was to know the clinical socio-demographic and investigational profile of unknown patients. Methods: We did retrospective chart review of all unknownpatients from January-2002 to December-2011 admitted under department of neurology National Institute of Mental Health and Neurosciences (NIMHANS) Bangalore. Clinical and socio-demographic characteristics of the sample were analyzed by descriptive statistics. Results: Total 151 unknown patients were admitted during 10 years period. Out of this 134(88.7%) were male mean age 43.8(14.8) years 95(63%) were aged more than 40 years 147(97.4%) from urban vicinity 126(83.6%) were brought by police 75(49.7%) were medico legal cases. Among these 3(2%) had normal sensorium 101(66.9%) presented with loss of consciousness. Deranged liver profile in 65(43%) renal parameters in 37(24.5%) dyselectrolytemia in 42(27.8%) and abnormal brain imaging in 95(62.9%) was found. 41(27.2%) had seizure disorder 37(24.5%) had metabolic encephalopathy and 26(17.2%) had stroke. Death (9.3%) or poor outcome (5.3%) was seen in 22 (14.6%) were expired. Complete recovery was seen in only 18 (11.9%) patients. Conclusions: Police brought majority of unknown patients to neurocasualty. These patients were in critical condition with altered metabolic profile and abnormal brain imaging. Poor outcomes were seen in a significant number. This study highlights the need of improving social responsibility and care in this group.
Cognitive impairment in SLE
Byju P, David, Robert Mathew
Pushpagiri Institute of Medical SciencesThiruvall, Kerala, India
Aim: To study the pattern of cognitive dysfunction in SLE Materials and methodProspective cross sectional study. 30 consecutive SLE patients attending a tertiary care teaching hospital during the period 2010 to 2012 were studied. Cognition was assessed using ACE-R. Trail A and B digit span and CBI. Results: All the patients were females. Mean age was 33.4(SD 9.53) years and education 2.07(SD0.83). Duration of SLE at the time of presentation was 50.93(SD 43.27) and duration of neurologic manifestation from onset of SLE 12.77(SD 17.045). Sixty percent of the patients had neurologic manifestations. Mean ACE score was 86.17(SD1.05) which was higher than the existing cut off score of 67. The ACE subscores were converted to percentage of maximum score for purpose of comparison and showed high values ranging from 63.57(SD9.06) for executive function to 90(SD 17.16) for visuospatial function. When one sample T test was used keeping 90 as test value significant difference in performance was seen between visuospatial function and executive function(p<.001) and language function(p<.001) and not other domains-memory(p=0.328) and orientation(p=0.856). Significant impairment in ACE score was seen in the group with neurolupus (p=0.053) when compared to others. When cognitive subscores were compared no difference was seen between the groups. Conclusions: In this cohort of SLE patients tests of cognitive functions appeared normal. However the performance in visuospatial function appeared poor when compared to performance in other cognitive domains. When compared with patients without neurolupus patients with neurolupus had significantly low ACE score.
Post Stroke Depression-A Cross Sectional Study
Elanthiraiyan G.C., Punya Mulky, Abid M, Thamil Pavai N, Lakshminarasimhan R, Bhanu K
Institute of Neurology Madras Medical College Ch, Chennai, Tamilnadu, India
Introduction: Post stroke depression (PSD) is the most frequent psychiatric complication of stroke with prevalence being around 20-60%. Patients with post stroke depression show far less recovery from functional impairments compared to stroke patients without depression. As a result these patients have a poorer quality of life. In our study we investigated prevalence of depression in patients after different types of stroke and compared it with their level of disability. Aim: To study the prevalence of depression in patients after stroke compare PSD according to age sex duration of illness site of stroke and etiology and find correlation between depression and level of disability. Materials and Methods: This study was conducted in Madras Medical College Chennai using a cross sectional study design.65 stroke inpatients from medical and neurology wards were included in this study. Instruments used for assessing severity of depression were Hamilton Depression Scale and Becks Depression Inventory-II. Modified Rankin Scale (MRS) was used to assess the disability encountered by patients. Results: In our study we found the prevalence of post stroke depression to be 65% it being more common in females (72.7%) as compared to males (55.8%). Prevalence is more in elderly age group (>60 yrs) (81%) especially in those with recent onset of stroke (<10 days) (77.7%). Left hemispheric stroke (71.8%) was more commonly associated with depression and those with higher disability score on MRS scored higher on the depression rating scales. Conclusions: We conclude that post stroke depression is more common in elderly patients females patients with left hemispheric stroke and higher disability scores.
Effect of Stress Management in Controlling Seizure Disorder
Praveen Chander, Fazal e ilahi, Kannan. V, K. Bhanu
Madras Institute of Neurology, Chennai, Tamil Nadu, India
Introduction: Among the various triggering factors for break through seizures in Indian population Stress plays an important role. Further the role of stress relief therapies in seizure control has also been addressed to. Aim: To study the effect of manipulation of stressors in controlling the seizure frequencies. Methodology: 75 epilepsy patients from our epilepsy clinic with >2 seizure episodes /month were included in the study. They were questioned regarding the stressors precipitating seizures. The stressors could be major or minor events as perceived by the patients. 44 patients could identify their stressors 5 had loss of loved ones 23 had family concerns 10 had occupational financial crisis and 6 had minor events like household commitments commutation difficulties. Excluding 5 patients with loss of loved ones the remaining 39 were counseled to cope up with their main stressor and were advised stress relieving modalities like meditation yoga and psychotherapy. These patients were reviewed after 3 months. Results: 29 patients (out of 39) on follow up had stress relief when compared to before. Among these 29 patients 20(68.9% p=0.09) (Outcome for each variables assessed by Fishers exact test) showed significant reduction of seizure frequencies (more than 50 % reduction in the preexisting frequency rate) 9(31.03%occupation/financial crisis-5family concerns -3 minor events group-1)had no clinical improvement. Conclusion: Stress reduction efforts by treating Physicians have a significant role in seizure control. Little extra time spent on advising stress reduction modalities in outpatient department can effect a better outcome in control of seizures.
Characteristics of nerve conduction studies in carpal tunnel syndrome
Parag Moon, Vijay Sardana, Dilip Maheshwari, Sankalp Mohan
GMC and allied hospitals Kota Rajasthan, Kota, Rajasthan, India
Background: Numerous nerve conduction tests are used for the electro diagnosis of carpal tunnel syndrome (CTS) with a wide range of sensitivity and specificity reported for each test in clinical studies. The purpose of this study was to compare the diagnostic accuracy of various nerve conduction tests and determine the properties of the most accurate test. Methods: 40 patients were enrolled in this study based on clinical characteristic of carpal tunnel syndrome. All patients were tested for hypothyroidism diabetes mellitus and rheumatoid arthritis. Then they were subjected to nerve conduction study protocols for CTS. Results: Mean age was 43.5 +/-13.68 yrs. Female: male ratio was found as 2.33:1. Mean duration of disease was 0.91+/- 0.76 yrs. Hypothyroidism was present in 16(40%) while 12(30%) and 3(7.5%) patients had diabetes mellitus and rheumatoid arthritis respectively. Median nerve motor latency was 4.18+/-2.01 ms while sensory latency was found as 3.00+/-0.76 ms. Median nerve orthodomic sensory latency was found as 3.24+/-0.75 ms. Conduction velocity across palm and wrist was 40.95+/-7.55 ms. Ulnar vs median nerve sensory latency was positive in 26(65%) while radial vs median sensory latency was positive in 24(60%) patients. Inching method was positive in 29(72%) cases. Conclusion: Probability of diagnosing CTS is more with inching method followed by ulnar vs median sensory latency.
Poster Session 14, Saturday, October 03, 2015,
02: 00 PM - 04:00 PM
Dr. Vimla Vermani Hall (Hall D):
Study on Association between Stress and Epilepsy
S. Fazale ilahi
Institute of Neurology, Chennai, Tamilnadu, India
Introduction: Stress is one of the most frequently self-identified seizure triggers in patients with epilepsy. This study explores the causal role of stress in triggering seizures in patients with existing epilepsy and scope for stress reduction therapies. Aim: To assess the relationship between stress and seizures in people with established epilepsy. Methodology: A questionnaire was administered to patients in our Institute of Neurology Chennai outpatient epilepsy department with >2seizures/month. Stress was assessed on the basis of major events (loss of loved ones debts job loss etc) and minor events(car traffic family tensions etc) and seizure frequency. Seventy five patients completed the questionnaire. Patients included in the study were: duration of epilepsy>1 year; age 18-60 all types of seizures and patients with low IQ were excluded. Results and Discussion: In our study 41 (54.6%) patients were female and 34(45.3%) were male. 44 (59%) out of 75 attributed stress to the increase in frequency of seizures. 5(11.36%) had loss of loved ones 23 (52.2%) had family issues 10 (22.7%) had financial and occupational issues 6(13.6%) had minor events. Among all the events family issues was principle precipitating factor for increased seizure frequency. Conclusion: Our study reveals that stress does play a role in increased seizure frequency and to improve better support systems like counselling psychotherapy and other stress reduction modalities in reducing seizure frequency.
Response of Brain Tuberculoma to Intermittent Antituberculous Therapy In Immunocompetent Patients: An Observational Study.
Thomas Iype, Ayyappan KJerin Cherian, Thomas Iype, Ayyappan K, Jerin Cherian, Chitra Pushpa
Government Medical College, Thiruvananthapuram, Kerala,India
The diagnosis of tuberculoma depends largely on multimodality MRI. At least some of the patients with tuberculoma are not completely cured with directly observed intermittent treatment for 9 month therapy as given in the Revised National Tuberculosis Control Programme (RNTCP regimen). Hence we wanted to see the response of tuberculoma with RNTCP regimen for 9 months. We prospectively recruited patients with brain tuberculoma of both sexes 13 years of age from January 1st 2010 for four and a half year period. Last follow up was on August 31st 2015. We excluded patients with associated HIV or other immunosuppressed conditions. All patients had MRI brain. We followed up all patients on RNTCP regimen with repeat MRI at 9 months as well as earlier when needed. We looked for disappearance of lesion paradoxical change in number or size gliosis and calcification. We also looked at the residual disability and death need for surgery and the time taken complete disappearance of the tuberculoma. Out of the 55 patients 45 had tuberculoma 4 had metastasis and 3 had glioma. Twenty one tuberculoma patients had meningitis. Tuberculoma patients had median age of 39 (2449) years majority of whom were female(26/45). Alcohol abuse in6/19 males. Seizure was presenting symptom in 17/45 patients. Complete clearnance at 9 months was seen in 19. Persistanc e of lesions at 2 years was seen in 11 patients. Calcification on follow up was seen in 5 patients. The commonAEDs used were clobazam and leveterecetam.
Circadian Profiles of Focal Epileptic Seizures - Role Of Chrono-Epileptology For Therapeutic Implications.
M. Jayasree,Dr. T. Suryaprabha, Sailaja Sarva, Dr. Subhash Kaul, Dr. Suvarna Alladi, Dr. Abhijeet Kumar Kohat
Senior Resident, Hyderabad, Telangana, India
Background: Time based distribution of peak incidence of seizure occurrence within 24-hr day cycle with certain epilepsy syndromes is demonstrated in studies. Circadian influences of epilepsy by phase shift of approximately 12 hours in 24 hour rhythm of hippocampal spikes in animal model of limbic epilepsy and elicits consideration of epilepsy on circadian rhythms. Aims: 1. To see whether there is any preference of time predilection on 24-hour cycle for focal seizures and its relation to gold standard dim light melatonin onset (DLMO) of circadian rhythm2. To see any circadian rhythm abnormalities in patients with epilepsy. Materials andMethods: 40 consecutive patients who attended presurgical evaluation of refractory epilepsy in Nizam's Institute of Medical Sciences were included in the study. Detailed clinical evaluation like semiology of seizures high resolution (1.5-3T) MRI brain and VEEG evaluation with seizure recording for localisation of lesion was done. The timing of onset of seizures in relation to 24-hr cycle and DLMO was recorded. 2 standardized questionnaire for circadian rhythm abnormalities (Morningness - Eveningness questionnaire(MEV) and Munich chronotype questionnaire (MCTQ) were used. Results were analysed. Conclusion: Temporal lobe seizures occurred more frequently 6 hours prior to DLMO frontal lobe seizures occurred mostly 6-12 hours after DLMO suggesting that seizure occurrence is synchronised to circadian rhythm phase in no random pattern.
Profile of Neurological Diseases duringPregnany and Puerperium: A Tertiary Care Centre Experience
Chaithra S P, Dr Chaithra S P, Dr Karthik N, Dr Archana N B, Dr J B Agadi, Dr Praveen Kumar, Dr D C Janardhan
Bangalore Medical College, Bangalore, Karnataka, India
Objectives: The aim of this study was to analyze the clinical and radiological spectrum of neurological complications in pregnancy and puerperium. Materials and Methods: This is a prospective analytical study conducted at a Tertiary care hospital in Bangalore from January 2014 to September 2015. Clinical data risk factors symptomatology imaging and outcome parameters were analyzed. Results: 86 patients were seen for neurological complications -Cerebral venous thrombosis- 12(14%)Posterior reversible encephalopathy syndrome-7(8%) Eclampsia-55(64%)1 case of cerebral infarct and haemorrhage each1 case of hypernatremic rhabdomyolysis 2 cases of neurocysticercosis 1 case of Tubercular Meningitis with hydrocephalus 2 cases of Carpal tunnel syndrome 1 case of frontal astrocytoma1 case of chorea gravidarum. Among patients with eclampsia 40 were antepartum and remaining were postpartum. Seizures (100%) were the predominant presentation followed by headache (40%) and blurring of vision(18%). Among 12 cases of CVT 10 were postpartum and rest antepartum. Headache (100%) and vomiting(75%)were the predominant presenting symptoms followed by seizures(58%) and focal neurological deficits(58%). 11 cases had superficial venous system involvement. All had a good outcome. Among cases of PRES presentation was with seizures (71%) headache (57%)blurring of vision (28%) and impaired consciousness(28%). Allof them had a good outcome. Conclusion: Neurological complications during pregnancy are varied and need urgent management but if treated early usually carry a good outcome.
Stroke Related Epilepsia Partialis Continua
Kavitha M, Ganesh kumar, Meikandan, Manoharan
Madurai, Tamilnadu, India
Epilepsia partialis continua is a former of status epilepticus of partial seizures where there is continous focal seizure with intact sensorium. Common cause is Rasmussen encephalitis in children and SOL or AVM in adults. This patient presented with epc following stroke. 64yrs old presented with continous left upper limb jerks clonic movements with normal sensorium present for more than 24 hours. He also had weakness of left upper and lower limb with left facial palsy. His Imaging showed acute infarct in right MCA territory. Patient was treated like status epilepticus with iv anticonvulsants and he responded to high dose of levetiracetam along with conventional anticonvulsants. This case is presented for the rare presentation of stroke as Epilepsia partialis continua.
Hereditary Spastic Paraplegia with Thin Corpus Callosum Due To Spg11 Mutation
Samir Patel, P K Sethi, Ish Anand, Anuradha Batra, Pooja Gupta
Sir Ganga Ram Hospital, New Delhi, India
Hereditary spastic paraplegia (HSP) is a heterogeneous group of familial neurodegenerative disorders characterized by progressive lower limb spasticity. SPG11-associated HSP which is characterized by a thin corpus callosum is a common and clinically distinct form that is linked to the SPG11 locus on chromosome 15. We report a case of young male with hereditary spastic paraplegia with thin corpus callosum due to SPG11 mutation.
Pattern Of Hospital Admission and Outcome In Parkinson's Disease: Multiple Co-Morbidities of One Disease
Birinder Singh Paul,Gagandeep Singh, Gunchan Paul, R Bansal, Monika Singla
Dayanand Medical College & Hospital, Ludhiana, Punjab, India
Background: We studied the causes for hospital admission course in hospital and their outcomes in PD population so that preventive measures if possible could be developed. Methods: We prospectively studied all patients with the known diagnosis of PD admitted to the hospital till date. Based on the reason for admission patients were divided into two groups admission due to causes related to PD or not associated with PD. The PD related admissions were further categorized into Group I: directly disease related causes and Group II- indirectly disease related causes. The clinical parameters age gender duration of disease H & Y staging co-morbid conditions treatment history and complications during the admission were noted. The primary outcome was mortality. The secondary outcome measures were duration of hospitalization (days) requirement for ICU need for mechanical ventilation and complications. Factors potentially affecting hospital admission and course were analyzed by comparing the two groups. Results: There were total 25326 admissionsof these 153 patients had the diagnosis of PD. 44 patients (28.7%) had direct cause 76 (50%) had indirect disease related cause and 33 (21.2%) were non-PD related admissions. The mean age was 68.5 + 9.9 years 102 males (66.7%). There were 17 (10.9%) deaths in our study. The indirect PD group II had significantly higher age (p= 0.0014) increased risk of ICU admission (p=0.011) longer duration of hospital stay (9.22 + 6.80; p=0.0001) and need for mechanical ventilation (p < 0.005) as compared to group I. Also there is a six fold increased risk of death in PD patients who are admitted to the hospital with indirect reason related to PD (p 0.035). There was no co-relation with respect to gender duration of disease and H & Y stage with either cause of hospital admission or outcome. Conclusion: As disease progresses the indirect reasons for admission becomes more troublesome than the initial motor complaints.
FDG-Pet as a Radiological Biomarker in Patients with Takayasu Artetis
Kannan V, Shankar B, Srinivasan Raman G, Lakshmi Narasimhan R, Bhannu K,
Madras Medical College, Chennai, Tamil Nadu, India
Objective: The purpose of this study is to evaluate the usefullness of FDG PET in early identification of aortitis in patients with Takayasu arteritis presented as young stroke. Background: Takayasu's arteritis is a chronic granulomatous, primary vasculitis affecting large vessels with 10% of the people present as young stroke. Clinically Asymmetric pulse made us suspect takayasu and diagnosis made by angiography which is gold standard. More recently, vascular FDG-PET is emerging tool for vascular inflammation. Methods: Total of 10 patients with young stroke having takayasu arteritis diagnosed according to American college of rheumatology criteria. Seven patients with Takayasu arteritis in the active stage(showing elevated level of general inflammatory markers), 3 patients with Takayasu arteritis in the inactive stage, underwent FDG-PET and the inﬂammatory vascular lesion was evaluated by using the standardized uptake value (SUV) of FDG accumulation as an index. Patient were treated with steroids/immunomodulators and a follow up FDG PET done after 1 month to monitor treatment response. Results: The FDG PET revealed intense FDG accumulation (SUV > 2.7) in the vasculature of 5 cases in the active stage of Takayasu arteritis. The other 2 patients in the active stage revealed weak FDG accumulation (2.3 > SUV < 1.2). No signiﬁcant FDG accumulation was observed in the patients with inactive disease (SUV < 1.2). Given the cutoff SUV is 1.3, the sensitivity of FDG PET analysis of Takayasu arteritis is 66.67%. Finally, FDG accumulation resolved with therapy in 6 active cases.
Conclusions: The FDG PET images in Takayasu helps in early detection, evaluate the disease extent, grading based on the standardized uptake value and idealy to monitor the treatment response
Bickerstaff's Brainstem Encephalitis in Children - A Case Series from a Tertiary Care Centre.
PA Mohammed Kunju, Anagha Bhagwat, Gisi Shibu, Rose Mary Lawrence, Sudhakar, Kalpana D, Mini S, Mary Iype
Govt. Medical College, India
Background: Bickerstaff's Brainstem encephalitis(BBE) is rare postinfectious immunemediated disorder. It is a localized (CIS) form of ADEM. The condition is relatively rare in children and there are very few reports in literature pertaining to pediatric population. We had a cluster of cases of BBE recently.
Materials and Methods: Case records of Children admitted to our hospital between 1.4.2014 and 15.8.2014 fulfilling the criteria OF BBE according Bickerstaff et al. (acute onset of opthalmoplegia, ataxia within four weeks of onset associated with, altered consciousness) were analysed. Alternative diagnoses like brain tumour, myasthenia gravis, poisoning were excluded by appropriate investigations.
Results: There were five cases fulfilling the criteria. Out of them three were females, two were males. Age range from 5 to 11 years. Besides ophthalmoplegia and ataxia disturbance of consciousness was frequent (80%), and facial palsy (60%), Babinski's sign (60%) and pupillary abnormality(40%) and bulbar palsy (40%) were present. One child presented with respiratory failure. Ocular flutter was seen in one. CSF changes were present in (40%) patients. MRI was showing hemorrhagic hyperintense lesions in the thalamus and brainstem and was normal in 4/5. EEG was abnormal in 40%. All patients received acyclovir. High dose of steroids were given to 4 patients, 4 children received IVIG and one underwent plasmapheresis. 2 patients required ventilation during the hospital course. Average hospital stay was 23 days. One child had a relapsing remitting course and is non ambulant, the ptosis and ophthalmoplegia is persistingin one. Other three had acomplete recovery. Conclusion: This case series is being presented to highlight the clinical and features of this rare entity, the high chance of imaging being normal and to highlight the favorable outcome to immunosuppressive therapy.
Poster Session 15, Saturday, October 03, 2015,
02: 00 PM - 04:00 PM
Dr. Vimla Vermani Hall (Hall D):
X-Linked Spinal and Bulbar Muscular Atrophy with Myasthenic Symptoms.
Manjesh Rathi, Suresh Kumar, Stephen S, SSK Ayyar, GKumaresan
Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India
We describe a patient with X-linked spinal and bulbar muscular atrophy Kennedy disease(X-SBMA) and myasthenic symptoms. X-linked bulbospinal muscular atrophy (X-BSMA) is a slowly progressive motor neuronopathy characterized by adult-onset weakness and atrophy of the proximal limbs and bulbar muscles. Although X-BSMA differs pathogenetically from myasthenia gravis (MG) X-BSMA can be occasionally misdiagnosed as MG due to their similar clinical manifestations including muscular weakness without sensory symptoms ease of becoming tired fatigue and bulbar weakness. This patient was characterized by the clinical symptoms of fatigability decremental motor responses to repetitive nerve stimulation and improvement of the myasthenic symptoms with oral administration of pyridostigmine. No serum antibody to acetylcholine receptor was detected. It is suggested that in the process of chronic denervation and reinnervation of X-SBMA reinnervated motor endplates may be associated with the defect of neuromuscular transmission. Key Words: Kennedy disease(X-SBMA) decremental motor responses myasthenia gravis (MG).
Acute Quadriparesis: GBS or Bilateral Medullary Infarct : A Neurologist's Dilemma
DilemmaLeny Thomas, Moncy Thomas, Stephen S, Suresh Kumar, Deepak Arjundas
Sree Balaji Medical College & Hospital, Chrompet, Chennai. India.
Background: Bilateral medial medullary stroke is a very rare type of stroke with catastrophic consequences. Early diagnosis is crucial. Patient and Methods: Here we report a case presenting with progressive generalized weakness of all 4 limbs and initially diagnosed a case of AIDP initially. Later on electro diagnostic and imaging studies confirm a diagnosis of bilateral medial medullary stroke. Brain magnetic resonance imaging (MRI) that was done showed characteristic "heart appearance" shape at diffusion weighted (DWI) and confirmed bilateral medial medullary stroke. Other possibilities were excluded by lumbar puncture and MRI of cervical spine with screening of whole spine. Retrospectively a vague-defined hyper intense linear DWI signal at midline was noted in the first brain MRI. Results: Symmetric and midline pattern of this abnormal signal and similarity to an artifact some radiologists or neurologists may miss this type of stroke. Radiologists and neurologists must co relate clinical and MRI findings of this rare type of stroke where early treatment could make a difference in patient outcome and therapy. The abnormal DWI signal in early stages of this type of stroke may not be a typical "heart appearance" shape and other variants such as small dot or linear DWI signal at midline must be recognized as early signs of stroke.
Rituximab in CIDP
Anish Ninan, Ramadoss K, R Balakrishnan, R Ravichandran, Kunal Jadhav
PSG Medical College, Coimbatore, Tamil Nadu, India
Mr. JP 38 years, presented on 15th February with weakness and numbness of both distal extremities insidious onset and progressive. Examination revealed areflexic paraparesis. CSF analysis and Nerve Conduction studies substantiated a diagnosis of AIDP was given 3 cycles of plasma exchange. No further progression of disease after admission and discharged. Readmitted on 20th March with neck muscle proximal and distal weakness of both upper limbs and lower limbs with bulbar weakness. MRI Brain was normal. Underwent plasma exchange no improvement. Sural Nerve biopsy done. Developed Aspiration Pneumonitis Sepsis DIC Ventilated. Treated with multiple antibiotics tracheostomy done on 20/4/2015. 1st dose of IV Rituximab given followed by 2nd dose a week later. Improvement of Neck Muscle power Limb power decanulation done on May 10th. Discharged on May15th could walk with minimal support take oral feeds and could speak well. Rituximub is a chimeric monoclonal antibody which target CD 20 on B-lymphocytes. It is well tolerated and has widespread use in the treatment of rheumatoid arthritis SLE Dermatomyositis. Rituximab is comparatively cost effective and may be considered as a treatment opinion for CIPD especially in developing nations like ours.
Study of Prolonged Exercise Testing in Periodic Paralysis and Guillain Barre Syndrome
Bhavesh Solanki, Atri Chatterjee, Pragateshnu Das, D.P. Chakraborty, Gautam Guha, S.P. Saha
NRS Medical College and Hospital, Kolkata, West Bengal, India
In periodic paralysis progressive loss of muscle fibre excitability can be demonstrated by decremental pattern of CMAP in prolong exercise testing while there has not been any data of such test in Guillain Barres syndrome. Objectives: (1) To study the result of prolonged exercise testing in our periodic paralysis population. (2) To study the response of prolonged exercise testing in GBS. Material and Methods: Baseline CMAP was recorded from abductor digiti minimi after supramaximal stimulation of ulnar nerve at wrist with hand in complete rest. Isometric exercise was done for 5 minute and CMAPs was recorded in between with 1 minute interval further CMAPs were recorded at interval of 3-5 minute till 50-60 minutes in 9 periodic paralysis and 6 guillain barre patients. Decrement of 30% was considered significant and abnormal. Results: 7 out of 9 periodic paralysis patients showed decrement while none of the GBS patients. Conclusion: In the absence of routine genetic testing exercise testing is good easy and cheap alternative to support the diagnosis of periodic paralysis. We are expecting to present statistically significant number of patients at the end of study and that will be presented and discussed.
Statin Associated Ocular Myopathy Mimicking Ocular Myasthenia: Rare Occurrence
Abhinandan KS, Rohan Mahale, mahendra javali, Anish Mehta, Aju abraham john, Kiran buddaraju, Madhusudhan BK, Suryanarayana Sharma, R Srinivasa
MS Ramaiah Medical College and Research, Bangalore, Karnataka, India
The 3-hydroxy-3-methyl-glutaryl-CoA (HMG-CoA) reductase inhibitors more commonly known as statins are the most widely used medications for decreasing low-density lipoprotein (LDL) cholesterol. Statin associated myopathy is defined as occurrence of symptoms of myalgia in addition to an elevation in serum creatine kinase (CK) greater than 10 times the upper limit of normal. The proposed mechanisms for statin associated myopathy are reduced sarcolemmal cholesterol isoprenoid depletion and ubiquinone or CoQ depletion. The involvement of ocular muscles in statin associated myopathy has not been reported. We report a middle aged lady on simvastatin who developed unilateral ptosis with external ophthalmoplegia sparing the pupil. Work-up for myasthenia gravis and cavernous sinus syndrome was negative. There was complete resolution of symptoms with cessation of simvastatin. This case depicts the rare occurrence of statin associated ocular myopathy mimicking ocular myasthenia.
Lipoma Causing Posterior Interosseous Nerve Palsy - A Case Report
P Chandra Shekar, Dhairyawan Pokalkar
Introduction: Lipomas commonly occur in the subcutaneous tissue. Rarely present in the deep soft tissue causes paralysis of the posterior interosseous nerve (PIN) because of its anatomical relationship in that area. Below the elbow the PIN passes between the superficial and deep layers of the supinator muscle. The proximal edge of the supinator muscle forms an arch the arcade of Frohse. The PIN is vulnerable in this region. We report a case of an intramuscular lipoma with compression of the PIN. Objective: To report a case of Lipoma causing posterior interosseous nerve palsy. Materials and Methods: A 48-year-old gentleman presented with insidious onset gradually progressing inability to extend the fingers of the right hand over 4 months. No history of trauma. Examination revealed a swelling of 4β€-β€2 cm in the anterolateral aspect of the right forearm just below the elbow. The swelling was soft in consistency immobile not fixed to the skinno signs of inflammation. The power at elbow wrist and flexor of the fingers were normal. Decrease in the extension strength of the metacarpophalangeal joints of the fingers and thumb. (power 1/5). There was no sensory deficit. Results: Motor nerve conduction studies (NCS) and Needle Electromyographic examination revealed denervation of the right posterior interosseous nerve. MRI right forearm suggestive of intramuscular lipoma. Conclusion: The prognosis of PIN palsy depends on an early diagnosis followed by an immediate surgical excision. After surgical excision rehabilitation is needed for rapid functional recovery.
A Rare Mitochondrial Disorder: Leigh Syndrome: A Case Report
Ravindran S, P.R. Sowmini, S. Sakthi Velayutham, K. Malcolm Jeyaraj, S. Gobinathan, K.S. Raja, K. Karunakaran, P. Sampath Kumar, A. Vignesh
Government Stanley Medical College Hospital, Chennai, Tamil Nadu, India.
Leigh syndrome (LS) or Sub acute necrotising encephalomyelopathy (SNE) is a rare inherited mitochondrial disorder of childhood. The clinical presentation of LS is highly variable. However, in most cases it presents as a progressive neurological disease with motor and intellectual development delay and signs and symptoms of brain stem and/ or basal ganglia involvement. The diagnostic criteria are 1. Progressive neurological disease with motor and intellectual developmental delay, 2. Signs and symptoms of brainstem and/ or basal ganglia disease, 3. Raised lactate levels in blood and CSF, 4. Characteristic symmetric necrotic lesions in the basal ganglia and/ or brainstem. The characteristic neuro-radiological findings are bilateral symmetric focal hyper intensities in the basal ganglia thalamus substancianigra and brain stem nuclei most commonly putamen. Here we report a 12year female child who presented to us with spastic quadriparesis slurring of speech gait disturbances and cognitive deficits which was progressive over a period of 2 years without any cranial nerve or autonomic impairment. The disease precipitated by an acute febrile illness which was associated with seizures. There were no neurocutaneous markers. Family members are normal. MRI Brain showed T2/FLAIR hyperintensity with no diffusion restriction noted in periaqueductal region and bilateral putamen. CSF lactate levels were elevated. Nerve Conduction Study done in all four limbs was normal. Opthal ENT and cardiac evaluation done on was normal. Treated with Thiamine (B1) Coenzyme Q 10 Carnitine. The case is being presented for its rarity and classical clinical radiological and biochemical features.
A Case Series of Hypokalemic Paralysis Mimicking GBS
V.P. Youmash, R.M. Bhoopathy, M. Jawahar, E Uma Maheswari
Tamil Nadu Government Multisuperspeciality Hospital, Madurai, Tamil Nadu, India
Introduction: Guillian Barre syndrome and hypokalemic paralysis are two important causes for acute flaccid quadriparesis with similar clinical scenario. Also the nerve conduction abnormalities of both conditions especially compound muscle action potential (CMAP) amplitude declines during a paralytic attack creating clinical dilemma in diagnosis and management. We report four cases of hypokalemic paralysis mimicking AMAN variant of GBS electrophysiologically who had severely reduced CMAPs in all motor nerves at presentation. However the amplitude of CMAPs increased and reached normal levels as the serum potassium concentration and motor power returned to normal state. Case Vignette: Four patients of varying age group were admitted with gradually progressing motor weakness of all four limbs. Initial workup showed reduced serum potassium and typical ECG features of hypokalemia. With GBS as close differential diagnosis Nerve conduction study done revealed reduced CMAP in most of motor nerve conduction resembling AMAN variant of GBS. All patients were treated with potassium infusion with regular ECG monitoring and serum potassium. Subsequent conduction studies in two or three days revealed dramatic improvement of CMAP potentials as well as increase in motor power and normalisation of ECG and serum potassium. Discussion: Hypokalemic paralysis and GBS differentiation is crucial as the prognosis and management of both differsCost of immunoglobulin therapy or plasmapheresis is much compared to easier correction of potassium through oral syrup or intravenous KCL infusion. Conclusion: This study series emphasizes the importance of simple measurement of serum potassiumbed side ECG and serial nerve conduction studies showing reversal of electrophysiological abnormalities helping in narrowing down the diagnosis of acute flaccid quadriparesis thereby aiding appropriate management of the ailment.
Non Hodgkin's Lymphoma Relapse as Neurolymphomatosis: An Atypical Presentation
Ritu Shree, Vivek Lal, Manoj Goyal
Post Graduate Institute of Medical Education and Research, Chandigarh, Punjab, India
Background: Non-Hodgkin's lymphoma (NHL) may directly infiltrate the central and peripheral nervous system at various levels. Bedside the leptomeninges malignant lymphocytes can infiltrate the neuromuscular junctions peripheral nerves and nerve roots. But the involvement of single peripheral nerve is very rare with only few cases reported in literature. Observation: We report a 68 year male with history of NHL in complete remission presented with right foot drop. Nerve conduction studies suggested right axonal sensorimotor peroneal and tibial neuropathy suggestive of sciatic nerve involvement. MRI thigh showed localised thickening of right sciatic nerve at mid-thigh level with normal study of lumbosacral plexus. FDG-PET showed avid nodular thickening in the right sciatic nerve. There was no evidence of systemic involvement. Conclusion: Relapse of NHL with isolated involvement of peripheral nervous system is very rare with limited case reports in literature. So while evaluating peripheral neuropathy in a patient of lymphoma we should always look for relapse of the primary disease. This index case highlights the importance of high suspicion and proper evaluation with MRI and FDG-PET in making the diagnosis of neurolymphomatosis.
Poster Session 16, Saturday, October 03, 2015,
02: 00 PM - 04:00 PM
Dr. Vimla Vermani Hall (Hall D):
Myasthenia Gravis in a Post Coronary Artery Bypass Graft (Post CABG) - Two Case Reports
Dar Maqsood Ahmad, Malik Ab Hamid, Saikia Vasundhara, Goel Khushbu, Kumar Mukesh, Mukherji JD
Mzx Superspeciality Hospital, Delhi, India
Background: Among the disorders of neuromuscular junction, myasthenia gravis is the most common one. It is a heterogeneous disorder with no specific etiology identified in about 90 percent of cases. However evidence in favour of genetic predisposition to the disorder has been found to exist which is precipitated by the environmental factors 1. There are only few case reports of myasthenia graves occurring in patients after cardiac surgery (post CABG) in the literature so far. Objective: To show the association of myasthenia graves with coronary artery bypass grafting (cardiacsurgery). Material and Methods: We are reporting two cases of myasthenia gravis presenting immediately after Coronary artery bypass surgery (CABG). Case1: 70 years old gentleman admitted with triple vessels disease. He underwent CABG successfully. He was extubated as per the post-operative plan but reintubated due to poor breathing response. He improved significantly after intubation. He was again extubated but similar situation continued. Then neurologist opinion was taken. Family members denied any past history of easy fatigability, ptosis or bulbar dysfunction. Examination revealed asymmetrical ptosis with bulbar dysfunction with proximal quadriparesis. Possibility of neuromuscular junction dysfunction was kept. Neostigmine test was positive. He was started on intravenous immunoglobulin and later continued on steroid and azathioprine. Serum anti-acetyl choline receptor antibody and anti-MuSK antibody were negative. He recovered completely. Gradually steroid was tapered and switched to azathioprine during OPD followup. Case2: 72 years gentleman admitted with triple vessels disease. He underwent CABG successfully. He was extubated as per the post-operative plan but reintubated due to poor breathing response. He developed sepsis and acute renal failure. Initially it was thought of encephalopathy related to multiple organ dysfunctions. Family members denied any past history of easy fatigability, ptosis or bulbar dysfunction. Examination revealed bulbar dysfunction with proximal quadriparesis. Neostigmine test was negative. He was started on intravenous immunoglobulin and later continued on steroid and azathioprine. Serum anti-acetylcholine receptor antibody and anti-MuSK antibody were negative. He was discharged in stable condition with improving bulbar dysfunction. He came again in triage with type 2 respiratory failure after stopping pyridostigmine. He was again advised for intravenous immunoglobulin. He later succumbed to sepsis with multi-organ dysfunction following prolong intubation. Discussion: We report two cases of myasthenia graves in patients few months after cardiac surgery (CABG). Although myesthenia gravis (MG) developing in a patient shortly after the cardiac surgery could be coincidental, but as the similar case reports have already been mentioned in the literature, it needs tobeseriouslyreported. 2. Myasthenia gravis and coronary artery disease have been associated for many years but specific etiological link between the two entities does not exist. Most of the existing data is in the form of case reports and case series. Scoppetta et al. has reported three cases of cardiac surgery patients who developed myasthenia graves within a few weeks after cardiac surgery. 3. Anti-acetylcholine receptor antibodies and the clinical symptoms of MG have been found to develop in mice after administration of AChR intraperitoneally. 4. The thymus contains myoid cells. It has been mentioned that the atrophic thymic remnants may be damaged during cardiac surgery. As these cells can express AChR molecules and their damage can cause the release of AChR, this can increase a subclinical response to the antibody. Presentation of AChR to the immune system during a relatively difficult postoperative period could be the other factor responsible. 5. These cases are unique in the way that they presented in immediate postoperative period and both are biochemically negative. Still they improved completely with immunomodulator therapy. These cases remind us about possibility of NMJ disorder or MG in bulbar dysfunction. Conclusion: Although the association between the myasthenia graves developing after the cardiac surgery is rare, it should be thought as a possibility in all cases of bulbar dysfunction without any other motor deficit.
A case Chronic Inflammatory Demyelinating Polyneuropathy associated with Hepatitis B virus Infection.
Saikia Vasundhara, Abdul Hamid Malik , Ahmed Maqsood Dar, Goel Khushbu, Kumar Mukesh, Mukherji JD
Max Superspeciality Hospital, Delhi, India
Background: Neurological manifestations of Hepatitis B infection are rare. Only few case reports showing association of HBV infection with Demyelinating Neuropathy (AIDP,CIDP) have been reported. The pathogenesis is not known but most likely it is immune complex mediated.(1,2) Objective: To show the association of Chronic Inflammatory Demyelinating polyneuropathy with HepatitisBinfection. Material and Methods: We report a single case of Chronic Inflammatory Demyelinating polyneuropathy with Hepatitis B infection. A 47 years old diabetic man was admitted in hospital with 10 days history of insidious onset rapidly progressive flaccid quadriparesis with bilateral facial and neck flexor weakness and jaundice. On neurological examination, he had quadriparesis, weak flexion at neck and bilateral lower motor neuron type 7th nerve palsy. Deep tendon reflexes were absent. He had jaundice but no asterixis. Serum Biochemistry revealed hyperbilirubinaemia, hypergamaglobulinaemia and elevated liver enzymes. HBsAg and Anti HbcAb were positive with raised HBV DNA levels. Immunofixation was negative for M-spike. Neurophysiological examination demonstrated demyelinating polyneuropathy. His MRI of Brain and whole spine were normal. He was diagnosed with Acute Inflammatory Demyelinating Polyneuropathy and given IVIG along with antiviral. Consequently his power in both lower limbs improved partially. His hepatic dysfunction improved on antiviral and was discharged. However after 7 days of discharge he was readmitted with history of progressive dyspnea and altered sensosium of 2 days duration. On examination his chest expansion and single breath count were significantly impaired and ABG showed type II respiratory failure with CO2 narcosis. He was intubated and put on ventilator and was managed in ICU. He was started on systemic steroids and Azathioprine. His neurological symptoms and ventilator requirement improved within 2 weeks. Result: Initially he was diagnosed with acute inflammatory Demyelinating Polyneuropathy which later progressed to Chronic inflammatory Demyelinating Polyneuropathy which responded to systemic steroidsandimmunosuppressants. Conclusion: Although it is rare, Chronic Hepatitis B infection can manifest with demyelinating peripheral neuropathies like AIDP,CIDP which respond to systemic steroids and immunosuppressants.
Posterior Reversible Encephalopathy Syndrome (Pres) Associated With I.V Methyl Prednisolone and Methylcobalamine
Abdul Hamid Malik, Ahmed Maqsood Dar, Saikia Vasundhara,Goel Khushbu, Kumar Mukesh, Mukherji JD
Max Superspeciality Hospital, Delhi, India
Background: Posterior reversible encephalopathy syndrome (PRES) is a novel clinical entity commonly presenting with headache, seizures and visual changes. Reversible complications like status epilepticus intracranial aemorrhage and ischemic infarction may also occur. The most accepted theory for pathaphysiology is failure of autoregulation leading to hypoperfusion and disruption of blood brain barrier leading to vasogenic edema and potential reversible ischemia. PRES syndrome associated with I.V methyl prednisolone and Vit B12 is a rare entity, the pathogenesis being not known exactly.51 years lady presented in casualy with severe anemia and paraparesis. Examination revealed severe anemia and paraplegia. Biochemically she had sever vitamin B12 and iron deficiency. MRI spine showed long segment myelitis in dorsal cord. She received intravenous iron, vitamin B12 and methylprednisolone pulse therapy alongwith supportive medications. After 5 days she developed behavioral changes followed by seizure. MRI brain showed bilateral diffuse white matter edema with faint restricted diffusion in cortical and subcortical region. Over all features suggest possibilities of posterior reversible encephalopathy syndrome (PRES). She further received pulse methylprednisolone and continued on dexamethasone. She recovered significantly. She developed acute deep venous thrombosis and started on anticoagulation.
In her last outpatient visit, she started standing with support and coherent and alert in all activities.
This case is unique as she developed PRES on vitamin B12 and methylprednisolone. Either of them may be the precipitating factor. There are occasional case report of PRES with methylprednisolone and vitamin B12. Near complete clinical recovery in a month on tapering steroid confirms possibility of PRES. Discussion: The first patient to develop PRES syndrome associated with methyl prednisolone pulse therapy was a six years old child with juvenile idiopathic arthritis1. The second case case of PRES syndrome associated with methyl prednisolone pulse therapy was reported by Faruk incecik, M. Ozlem Herguner et al. 2 in a 8 year boy who presented with fever and pallor for 2 days and was diagnosed as Idiopathic Auto immune Hemolytic Anaemia. The patient was transfused with packed red cells and i.v methyl prednisolone at a dose of 30 mg /kg body weight was given started. On day 4 the patient suddenly developed headache,confusion and seizures and was diagnosed as PRES Syndrome. PRES Syndrome has also been seen to be associated with oral high dose corticosteroids. Sarah A. Morrow, Robina et al. reported the first case of PRES syndrome secondary to high dose corticosteroid 1250 mg of oral prednisolone for 5 days for the treatment of acute attack of multiple sclerosis relapse. 3 PRES syndrome has also been seen to occur due to use of high dose oral as well as i.v corticosteroids used for many indications like SLE, multiple sclerosis,auto immune hemolytic anaemia etc. Conclusion: Take home message of this presentation is that the possibility of PRES syndrome while using i.v methyl prednisolone and Vit B12 should be kept in mind, as any of the two could be a contributing factor for PRES syndrome and early treatment can reverse this condition ,thus emphasizing the need for early diagnosis and treatment.
Profile on Status Epilepticus in Children In ICU
Aim: To Study Clinical Profile on Status Epilepticus, Lab Parameters and outcome of the Children Admitted in Pediatric Intensive Care. Materials And Methods: STUDY POPULATION: Children between 1 month to 18 years who were fulfilling the definition of Status epilepticus Inclusion Criteria:
Children fulfilling definition of Status epilepticus
Age group 1 month to 18 years
Children admitted in PICU for Status epilepticus
Comprehensive Clinical and Treatment Outcome Profile of Patients with SUNCT and SUNA: A Tertiary Care Headache Clinic Based Study from North India
Debashish Chowdhury,Ruby Chopra, Soumya Sharma, Geeta Khwaja, Sanjay Pandey
GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, Delhi, India
Background: No systematic study is available from India regarding short lasting unilateral neuralgiform headache attacks with conjunctival injection & tearing (SUNCT) & short lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA). Objective: To assess and analyze headache characteristics, premonitory symptoms, triggers autonomic and behavioural features, systemic & psychiatric co-morbidities & treatment outcomes at 3 months in patients with SUNCT and SUNA. Methods: Patients attending the Headache clinic from August 2013 to December 2014 were diagnosed prospectively using ICHD 3 Beta classification and assessed by a detailed standardized protocol. All underwent MRI (Brain) to exclude secondary causes. Standardized treatment outcomes were categorized as complete remission, significant improvement (more than or equal to 50% reduction in attack frequency) & failure (less than 50% reduction in attack frequency). Ethical clearance was taken. Results: Out of 1084 headache patients, sixteen patients (1.5%) of SUNCT & SUNA (8 each), 11 episodic and 5 chronic (mean age of 43.06 13.18 years & M: F of 1.28) were seen. Attacks were witnessed in all patients. Mean symptom duration (4.65 years), VAS pain intensity (8.65), attack duration (185.6 seconds), attacks/day (38.06) were noted. 100% had side locked headache (right: left ratio 2.2). Locations included temporal (87.5%) & supraorbital (68.75%). Pain was electric current like (68.8%), throbbing and piercing (18.8%) & pricking (6.25%). Autonomic symptoms/signs were present in 100% patients. All SUNCT patients had conjunctival injection and tearing during attacks whereas in SUNA tearing was seen in 50%. Other autonomic symptoms included eyelid edema (43.6%), aural fullness (31.3%), facial sweating (31.3%), flushing (18.8%), nasal congestion (12.5%), rhinorrhea (6.3%) & ptosis (6.3%). 100% patients had restlessness during attacks. These included rubbing head (93.75%), changing position (87.5%), crying (68.8%), pacing around (56.3%), shouting (37.5%) & banging head (31.3%).13 patients (81.3%) had suicidal ideations, 56.3% had panic attacks while 37.5% had impending doom during attacks. 12.5% patients had premonitory symptoms. Cutaneous triggers (94%) included touching/washing face, chewing, eating, talking, brushing teeth/hair, bending/rotating head, shaving and external pressure against C3-4 roots (18.8%). 75% patients experienced allodynia. 50% patients had associated systemic co-morbidities like hypertension, hypothyroidism, diabetes, cardiac disease & history of head injury. 12.5% patients had concomitant trigeminal neuralgia. 15 patients (93.8%) were misdiagnosed previously. On treatment at 3 months, none remitted completely, 13 patients (81.3%) had significant improvement & 3 (18.8%) did not respond adequately. Conclusion: SUNCT & SUNA were misdiagnosed and average delay in diagnosis was 4.7 years. Strict unilaterality, autonomic features, restlessness, triggers, & multiple short duration attacks (seconds to few minutes) were the chief characteristics. Compared to Western data, we found rubbing the affected area of the head is a typical feature during the attacks and suicidal ideation is as common as in cluster headache. Great premium is attached to the correct diagnosis as majority of patients showed significant improvement with treatment.
Rasmussens Encephalitis-Rarest Of the Rare
Rahul Nag, U. Meenakshisundaram, C.U. Velmurugendran, Shankar. V, Prabash. PR
Sri Ramachandra Medicaluniversity, Chennai, Tamil Nadu, India
Introduction: Rasmussens encephalitis known as chronic focal encephalitis is a devastating disease usually occurs in children under 15years of age and affects one in 5 to 10lakh population and is characterised by progressive unihemispheric affection resulting in medically refractory focal seizures and contralateral neurological deficits. The disease predominantly affects healthy children and progresses variably through a prodromal,acute phase and a residual burntout phase. Although it is strictly unihemispheric disease,there have been very rare occurrences of bilateral disease. Methods: We report a case of Rasmussen encephalitis in a 8 year old boy in Chennai and to the best of our knowledge,is one among the few cases with this condition in Chennai. Third born child of non consanguinous parents with h/o febrile seizures at 2years of age , global developmental delay presented to us with involuntary jerky movements of right lower limb then gradually progressed and involved the whole body for which he was evaluated with MRI brain which showed atrophy of both the hemispheres(right more than left)with EEG showing bi-hemispheric dysfunction that involved temporoparietal and occipital lobes predominantly from the right side and we diagnosed the casebased on clinical findings,brain imaging,EEG according to European consensus criteria(partA)
Conclusion: We want to emphasize the importance of clinical examination,brain imaging,eeg in the diagnosis of seizure disorder,especially when it is intractable. To diagnose rasmussens encephalitis,the index of clinical suspicion is to be high.
Parkinsonism in Possible Normal Pressure Hydrocephalus
Pushpagiri Institute for Medical Sciences,Tiruvalla, Kerala, India
Aim: To study the Parkinsonism features in patients with Possible Normal Pressure Hydrocephalus. Method: Prospectivecross sectional study setting: Patients availing dementia care services from a tertiary care teaching centre were studied. Methods and Results: Fourteen patients with pNPH were included in the study. They underwent imaging, Addenbrookes Cognitive Examination (ACE), assessment of Parkinsonism using UPDRS and Cambridge Behavior Inventory (CBI) assesment. 50% were males, mean age was 76.06(SD6.39)years , mean duration of illness 26.14(SD18.73). Mean ACE score was 43.25(SD22.19). The mean UPDRS score was 22(SD2.71),range 0-94 thereby indicating mild impairment. 12(86%) patients had at least one UPDRS item impaired. Memory, behavior and mood impairment was seen in 7(43%), ADL in 9(64%) and motor impairment in 11(79%). Of the motor scores maximum number had impairment in leg agility 10(71%) and minimum had impairment in action tremor 2(14%). The maximum number impaired in ADL score was in cutting food 7(50%) and least impaired in 'turning in bed' 2(14%). When the individual UPDRS was analyzed minimum impairment was for sensory symptoms (mean score 0.14) and maximum leg agility (1.5). Minimum motor score was for action tremor (mean0.21) and maximum for leg agility. No correlation was seen between ACE composite score and UPDRS (p= 0.172). Negative correlation was seen between ACE sub scores and UPDRS sub scores (pearson's correlation p<0.05) which was not seen when controlled for age, sex and duration of illness. Conclusions: Parkinsonism appeared very common in NPH motor scores being more impaired. Impairment appeared mild even when the cognitive impairment was significant. Parkinsonism and cognitive impairment did not correlate.
A Study Of Psychiatric Co-Morbidities In Cluster Headache: Much More Common Than Previously Thought
Ruby Chopra, Debashish Chowdhury, Soumya Sharma, Geeta Khwaja, Sanjay Pandey
GB Pant Institute of Post Graduate Medical Education and Research,Dellhi, India
Background: Psychiatric co-morbidities in cluster headache (CH) have received lesser attention. These may have important bearing in their management & quality of life & hence need to be explored.
Aim: To assess the occurrence & spectrum of psychiatric co-morbidities in CH patients. Methods: CH patients in cluster periods during headache free intervals were prospectively screened for psychiatric co-morbidities by patient health questionnaire followed by detailed instruments for rating specific psychiatric disorders namely Hamilton rating Scale for depression, Generalized anxiety disorder-7 for anxiety, Yale-Brown obsessive compulsive scale for obsessive compulsive disorder,Mood Disorder Questionnaire for bipolar disorder & Anxiety sensitivity index-3 for panic attacks. Ethical clearance was taken. Results: Forty two CH patients (30 ECH and 12 CCH) with mean age of 38.3 years & male to female ratio of 2.2 were seen from August 2013 to May 2015. All ECH patients were in cluster period. Average symptom duration was 4.59 years. Mean pain intensity was 9.04 on CH rating scales. 20 patients (47.6%) had psychiatric co-morbidity. Specific psychiatric disorders seen were major depression (n=16; 38%), generalized anxiety (n=14; 33.3%), panic attacks (n=8; 19%) & somatization disorder (n=9; 21.4%). 30.9% patients (n=13) had both depression & anxiety. Suicidal ideation was present in 57.1% patients (n=24); 3 attempted suicide. 47.6% patients (n=20) had perception of impending doom during attacks. One patient (2.3%) had obsessive compulsive disorder (extreme cleanliness). None had bipolar disorder. Conclusion: Our study, the first from India on this subject shows that co-morbid psychiatric disorders are common in CH patients during cluster period, depression & anxiety being the commonest. These need identification & treatment for optimum outcome.
Clinical Features of Huntington's Disease and Correlation of Clinical Features with Genetic Study in Tertiary Care Centre in Eastern India
Introduction: Huntington's disease HD is a progressive degenerative disorder of the central nervous system with autosomal dominant inheritance characterized by involuntary movements cognitive impairment and behavioral disturbances. HD is associated with an expanded sequence of CAG repeats on the IT15 gene that is located on chromosome 4. Materials and Methods: Herein we report clinical data on 20 genetically confirmed HD patients from 17 Indian families predominantly from North East India. Clinical data and evaluation was performed using standardized formats used by the Huntington Disease Study Group. Results: Adult onset HD was commonest while. Juvenile HD onset - 20 years was observed in ; 10% of patients. Chorea was the commonest presenting symptom (n50 85%) while remaining presented with psychiatric symptoms (n7 15%). Impairment of saccades was observed in 75% of patients. Mean (SD) CAG repeats in the abnormal allele was 47.8 (8.7). Total motor score but not the total behavioral score worsens with duration of symptoms. The functional checklist score correlates with total motor score rather than with duration of symptoms. Conclusions: We detail clinical characteristics in genetically confirmed HD patients from a predominantly North East Indian cohort. We observed a slightly higher occurrence of Juvenile HD. Functional disabilities in our patients correlate with worsening of motor rather than behavioral symptoms.
Spectrum of Autoimmune Encephalitis at a Tertiary Care Centre
Vikram Asturkar, SP Gorthi, Pawan Dhull, YS Sirohi, Manoj S
Army Hospital R&R, Delhi, India
Autoimmune encephalitis is a well-recognised entity now and the spectrum of this disorder is widening with the detection of various antibodies. Early index of suspicion is required for diagnosis as the treatment results in good outcome. Aim: We conducted this prospective study to determine the spectrum of autoimmune encephalitis. Settings and Design: This is a prospective observational study conducted at a single centre tertiary care hospital. Methods and Material: We included eight patients presenting with various neurological manifestations with central and peripheral nervous system involvement. The patients were diagnosed with autoimmune encephalitis based on clinical findings and specialized investigations (autoimmune work up) as necessary. Results: The spectrum of autoimmune disorders ranged from NMDA encephalitis with predominant CNS involvement to Bickerstaff encephalitis with brainstem and peripheral nervous system involvement. The study included two patients with NMDA encephalitis three patients with steroid responsive encephalitis one patient of VGKC encephalitis and two patients of Bickerstaff encephalitis. Six of the eight patients responded well to treatment. The outcome was fatal remaining two cases. Conclusion: High index of suspicion is necessary in diagnosis autoimmune encephalitis as the prognosis is good if early treatment with immunomodulator therapy is initiated.
Poster Session 17, Saturday, October 03, 2015,
04: 00 PM - 06:00 PM
Dr. Vimla Vermani Hall (Hall D): Stroke
Study of Clinicoradiological Profile and Treatment Outcomes In Anterior Circulation Stroke Cohort In South India
Surya Narayana Sharma P M, Mahendra J V, Madhusudhan B K, Rohan R Mahale, Anish Mehta, Aju John Abraham, Acharya P T, Srinivasa R
M.S. Ramaiah Institute of Neurosciences, Bangalore, Karnataka, India
Background: Anterior Circulation Stroke (ACS) is defined as a symptomatic infarct in the anterior or middle cerebral artery territory or both. Objective: To study the epidemiology clinicoradiological profile and clinical outcomes in patients with ACS. Material and Methods: Study included patients with ACS at a tertiary care stroke program between October 2012 and September 2014. Patients with ACS above the age of 18 years developing stroke within 7 days of onset were included. A total of 310 patients were included. Results: There was a male preponderance (65.1%). Mean age was 61.5 years. One fifth of the patients were under 45 years of age. 21.29% of patients presented within window period. Right hemiparesis was the commonest clinical presentation (42.58%). Hypertension was the commonest medical comorbidity (58.7%) followed by diabetes (42.58%). 32(10.32%) patients had atrial fibrillation. Mean NIHSS score was 9.01. Left MCA infarct was seen in 133 (42.9%) patients. Stroke of undetermined etiology was the commonest stroke subtype (33.54%) followed by Large artery atherosclerosis (22.58%). 42(13.55%) patients were thrombolysed. Mean mRS at discharge was 2.42 and 1.45 at 3 months. Good outcome was seen in 187 (60.32%) and 232 (74.84%) patients at discharge and 3 months follow up respectively. There were 10 deaths (3.22%). Conclusions: Younger age male sex short window period and duration of ICU stay was associated with good outcome. There was a positive trend towards improvement in thrombolysis group (mRS: 1.31) versus general group (mRS: 1.47) at three months though it was statistically insignificant.
Protein S Deficiency And Arterial Ischemic Stroke
R Ramya, V Ramakrishnan, V Chandramouleeswaran, K Bhanu
Institute of Neurology Madras Medical College, Toruppur, Tamilnadu, India
Introduction: Protein S is the vitamin K dependent co factor for activated protein C which inhibits the coagulation cascade by inactivating factor Va and VIIa. Hereditary protein S deficiency is associated with familial venous thrombosis. But the relationship between protein S deficiency and arterial thrombosis is uncommon. However isolated case reports of protein S deficiency and cerebral arterial thrombosis withoutvascular risk factors have been documented. Studies have shown that in protein S deficiency around 1-5% cases are associated with venous thrombosis and <0.5% with arterial thrombosis. Case Series: Here we are presenting a series of 3 cases of ischemic stroke one presented with Left MCA infarct one with Right MCA acute infarct with multiple old infarcts and one with posterior circulation TIA and Right ICA complete occlusion. All of our patients are aged < 45 years males had decreased protein S levels with normal protein C anti thrombin III and other coagulation profiles. At present we have no scientific explanation why these patients developed arterial thrombosis than venous thrombosis. Possibilities include additional predisposing factors like male sex trauma cigarette smoking etc. Studies have shown smoking may precipitate and accelerate vascular events in protein S deficiency. Conclusion: Though rare protein S deficiency may present with arterial ischemia. The evaluation of young arterial stroke with predisposing factors to include Protein S deficiency as a routine evaluation and further management with anticoagulants/anti platelets at the first insult itself needs further studies.
Chilhood Moya Moya disease and Moya Moya syndrome A case series.
Santhosh Kumar B, Dhairyawan Pokalkar, Veena Narisetty, Sateesh Kumar, Sowjanya, Srilatha, Amareshwar Rao, Manjusha. B, Chandrasekhar
Gandhi Medical College Hyderabad, Hyderabad, Telangana, India
Introduction: MoyaMoya disease is a progressive stenosis of bilateral supraclinoid ICA with concomitant formation of tortuous arterial collateral vessels at the base of brain. The term β€moyamoya syndromeβ€ has been used to refer to cases that have angiographic features similar to those of moyamoya disease and causative systemic diseases which include sickle cell disease neurofibromatosis type 1 Down disease and other diseases. Objective: To report 3 cases of childhood MoyaMoya disease and syndrome. Materials and Methods: All patients were evaluated clinically along with MRI brainMRA and doppler of carotids Case 1: A 13yr boy with Downs syndrome presented with status epilepticus f/b right hemiparesis. CASE2: 18 months old girl presented with GTCS f/b right hemiparesis. Case 3: 3yr old girl sibling of case 2 also had a H/O right hemiparesis. Results: MRI: CASE1- Acute and subacute infarct in bilateral MCA territory. CASE2 - Acute infarct in bilateral MCA territory. CASE3 Chronic infarct in left MCA territory. MRA: CASE1 - Narrowing of bilateral cavernous ICA. Multiple collaterals noted around midbrain in perimesencephalic cistern collateral flow puff of smoke appearance - MoyaMoya pattern. Case 2&3 Narrowing of M1 segments of bilateral MCA with multiple collections around circle of Willis suggestive of MoyaMoya disease. CAROTID DOPPLER: CASE1 - RIGHT ICA grade III plaque from carotid bulb to proximal ICA causing 60% stenosis. LEFT ICA: 50 % stenosis. CASE2 RIGHT 50% & LEFT 70% ICA stenosis. Conclusion: The clinical and radiological features of MoyaMoya Downs syndrome overlap with primary Moya Moya disease β€"a protein encoded on chromosome 21 may be related to pathogenesis of MoyaMoya disease. The presence of MoyaMoya syndrome should be considered in the evaluation of Downs syndrome patients who present with neurological symptoms and signs.
Cerebrovascular Manifestations Following Scorpion Sting
N. Dinesh Kumar
Tirupathi, Andhra Pradesh, India
Introduction: Scorpion sting is a major public health problem in rural parts of India. Scorpion envenomation results in mild local pain to systemic manifestations. In this we report 2 cases of cerebrovascular manifestations following scorpion sting; one ischemic stroke and one hemorrhagic stroke that occurred in rural parts of Andhra Pradesh. Case Details: Case 1: We report a 25 year old female who had Scorpion sting on her foot she developed severe breathlessness diagnosed as myocarditis with moderate LV dysfunction. On the 3rd day after discharge she developed left hemiparesis and dysarthria with acute infarct in right capsuloganglionic region. She was managed with mannitol aspirin and supportive therapy and she recovered well. Case 2: A 32 years old female had red Scorpion stingfollowing which she developed severe myocarditis. On the 4th day of admission she developed drowsiness left 3rd nerve palsy and right hemiparesis. CT scan brain showed bleed in left frontal region with compression on the midbrain. She was treated symptomatically improved over 10 days. Conclusion: Scorpion venom is a neurotoxin acts on respiratory center vasomotor center nerve terminals and end pates of both striated and non striated muscles causing autonomic storm. This case report highlights the importance of early hospitalization suspicion of neurological complications and appropriate treatment measures for a better outcome.
Two Uncommon Infectious Causes of Cortical Venous Thrombosis
A Harshavardhan, Savitha P, Dhanaraj M
Apollo Main Hospital, Chennai, Tamilnadu, India
Introduction: Cortical venous thrombosis (CVT) is known to occur with several systemic illnesses and infections. Malaria and chicken pox are common tropical infections however CVT is rarely reported in them. In this presentation we report two cases of CVT one each following plasmodium vivax malaria and chickenpox. Case 1: A 54 year old male presented with alternate day fever and headache for 5 days. Clinical examination showed no focal neurological deficit. MRI brain with MRV showed thrombosis of superior sagittal sinus left transverse sinus and sigmoid sinus. Peripheral smear showed plasmodium vivax. He was also found to have raised anti cardiolipin antibody hyper homocystinemia and low protein C levels. Rest of the thrombophilia screen was normal. He was treated with antimalarials and anti coagulants with good clinical improvement. Case 2: A 20 year old male presented with 3 days of headache followed by 2 attacks of generalised tonic clonic seizures while he was recovering from chicken pox infection. Clinical examination showed chicken pox rash all over the body with no focal neurological deficit. MRI Brain revealed left temporal hemorrhagic venous infarction and MR venogram revealed thrombosis of left transverse and sigmoid sinus. He was also found to have hyper homocystinemia raised anticardiolipin antibodies with weakly positive lupus anticoagulant. Rest of the thrombophilia screen was normal. He was treated with anti edema measures Acyclovir anticoagulants and anti epileptics with good clinical improvement. Conclusion: The above two are very uncommon causes of CVT following common tropical infections. The possibility of CVT should be kept in mind in such cases of tropical infection where the headache is very severe and persistent.
A Study on the causes for Nonthrombolysis in Stroke Patients
Ulagaratchagan. V, Kannnan. V, Bhanu. K
Madras Institute of Neurology, Chennai, Tamil Nadu, India
Aim: To assess the causes for delay in thrombolysis in stroke patients admitted in Madras Medical College. Materials and Methods: This was a descriptive study done on stroke patients admitted in the casualty medical and neurology wards of Madras Medical College based on the information collected from the patients and their relatives. Results: The study was done on 30 stroke patients. Ischemic stroke 25 (83.33%) hemorrhagic stroke 5 (16.67%). 7 (23.33%) of them <40yrs 17 (56.67%) of them were 40 to 65yrs 6 (20%) were >65yrs. Travel distance <10km 2 patients (6.67%) 10 to 50km 15 (50%) >50km 13 (43.33%). Time of arrival <4.30hrs 3 (10%) 4.30 to 24hrs 15 (50%) >24hrs 12 (40%). Neuroimaging done in <6hrs 7 (23.33%) out of which 6 were ischemic stroke diagnosed outside >6hrs 23 (76.67%). In <4.30hrs time of arrival groups neuroimaging was done after 7hrs only. Even in <10km distance only 1 out of 2 able to reach hospital by 3.30hrs neuroimaging was done at 7.30hrs only due to financial issue. Conclusion: In the 25 ischemic stroke cases none of them were thrombolised due to delay in arrival referral delay in neuroimaging due to financial issues.
Arterial and Venous Thrombosis Associated With Heterozygous Factor V Leiden Disorder And Hyperhomocysteinemia: Importance Of Synergistic Factors In Two Cases With Stroke In Same Family
Manjesh Rathi, Suresh Kumar, Stephen, S.S.K. Ayyar
Sree Balaji Medical College and Hospital, Chennai, Tamilnadu, India
We described two cases of acute stroke in a family. A 27-year-old man with heterozygous factor V Leiden disorder presented with cerebral venous thrombosis (CVT). He suffered an episode of DVT one year back he was managed and started on long term oral anticoagulant therapy which he discontinued after two months. His father aged 62 years had a history of an arterial thrombotic stroke three year back investigation showed an activated protein C resistance due to factor V Leiden mutation and hyperhomocysteinemia. Homocysteine levels were normal in son at presentation. He showed no other vascular risk factors. Heparin was used to treat the CVT; long-term treatment included anticoagulation folic acid and risk factor modification. Although factor V Leiden is strongly associated with deep venous thrombosis additional cofactors such as hyperhomocysteinemia may predispose to an increased risk of acute arterial thrombosis in synergistic manner.
Key word: Factor V Leiden homocysteinemia CVT-cerebral venous thrombosis DVT- deep venous thrombosis.
Predictors Of Outcome of Intracerebral Hemorrhage Along The Sea Coast Population of South India
Anil Kumar. T, N.S. Sampath Kumar, M. Rajaguru, Ravi kiran. P, Ganesh. V, Sunanda T
Nellore, Andhra Pradesh, India
Introduction: Intracerebral hemorrhage (ICH) is second most leading cause for stroke followed by ischemic stroke which leads to high rates of mortality and morbidity. Despite advances in medical and neurosurgical treatment modalities ICH remains same with poor outcome with high rates of mortality projected worldwide up to 40% to 50%. Identification of major governing factors demonstrating clinical presentation and outcome of ICH is essential and also important in analyzing each population. Aim: To assess major governing factors among seacoast population presenting with ICH and their outcome. Materials and methods: Present prospective study was carried in Neurology department Narayana Medical College Nellore for a period of 1.5 years. A total of 158 patients are assessed for outcome of ICH among sea coast population. Results: In this study HTN is one of the most important modifiable risk factor in patients with ICH. With a mortality rate of ICH was almost 50%. Multivariate analysis of variables revealed that patients with pupillary asymmetry Left Ventricular Hypertrophy in 2D Echo High systolic blood pressure and low GCS (Glasgow coma Score) at presentation and CT (computerized tomography) imaging brain (plain) studies showed high peri-hematomal edema irregular hematoma border are major key factors associated with high rates of mortality Conclusion: Present study signifies that most seacoast population presented with ICH were illiterates and under low economic status. Hence life style modifications and health education are very important preventive key factors to reduce incidence and mortality of ICH among sea coast population by primarily focusing on primary prevention of hypertension.
Usefulness of Transesophageal Echocardiography In Stroke
Vikram Kishore Reddy P, K. Shiva Ram Krishna, Shailesh, Bimal Prasad, H Radha Krishna
Care Hospitals, Hyderabad, Telangana, India
Introduction: Aorta can be source of embolic stroke and study of aorta can sometimes give us insight into the atheromatous process going on in the vascular tree. A source for emboli can be the left atrium and its appendage. TEE is also useful in studying mitral valve apparatus interatrial septal identification and measuring patent foramen ovale aneurysmal atrial septum and myxoma of left atrium. Objective: To study findings of transesophageal echocardiography in ischemic stroke patients. Materials and Methods: The cases are collected from the records of Care Hospital Nampally Hydeabad from ischemic stroke patients. The present study includes a total of 500 such TEE records. Results: Male: Female ratio was 2.6( males = 362 females= 138). Different grades of abnormalities were seen in the aorta more commonly in the arch and descending aorta. The atrial septal abnormalities were seen in 18 cases. Clots or masses within the atria or ventricles were seen in 22 situations. The mitral valve was more often diseased in ischemic stroke patients though aortic valve disease or even multiple valve disease was also seen in many patients and the valve disease was of different grades. Conclusions: In present study it was useful in identifying an embolic source in 10% of patients and helped in initiating anticoagulation. It was also useful in identifying complex aortic atheroma in 18.4% cases (92 patients) and helped in starting different antiplatelet drug combination or anticoagulation. TEE helped in identifying the pathogenetic mechanisms of strokes.
Clinical Profile of Etiological and Risk Factors In Young Stroke
G Sree Rangalaxmi, S. Narmadha, Ramesh, Archana
Osmania Medical College, Hyderabad, Telengana, India
Aim of The Study: 1. To study about the clinical profile of stroke in young.2. To know about their risk factors and the etiology. Materials And Methods: This is the prospective study conducted in the age group between 1 and 18 years from September 2013 to June 2015 presented with stroke who are attending outpatient clinic at Osmania government general hospital Hyderabad. Consent were taken. Patients age sex detailed history were taken. Detailed examination was done. Investigations like hemogram electrolytes ECG 2D ECHO imaging CSF analysis Protein-C and S Antithrombin III and connective tissue profile were done. Data analysed and tabulated. Observation and Results: Totally 54 children were studied. Among them 23cases were due to hypercoagulable states 21 cases were due to infectious etiology 3 cases due to SLE 2 cases due to Intracranial bleed 3 cases due to cardiac cause 2 case due to metabolic cause. (1 case due to mitochondrial and 1 case due to Diabetes).
Poster Session 18, Saturday, October 03, 2015,
04: 00 PM - 06:00 PM
Dr. Vimla Vermani Hall (Hall D):
Clinico- Radiological Profile of Tubercular Meningitis- An Experience at a Tertiary Care Centre
Shaman Gill, SP Gorthi, VikramAsturkar, PawanDhull, Manoj S
Army Hospital R&R, New Delhi, Delhi, India
Clinico- radiological profile of tubercular meningitis- an experience at a tertiary care centre. Background and Aim: Tuberculous meningitis (TBM) is a life threatening form of CNS tuberculosis with a high morbidity and mortality despite treatment. The diagnosis is based on clinical radiological and laboratory features. We aimed to study the clinical and radiological profile of the patients presenting to our centre with tubercular meningitis over last 18 months. Materials and Methods: Diagnosis of TBM was based on clinical and laboratory and radiological features. Clinical imaging and laboratory features of 30 patients were studied retrospectively. Results: The commonest clinical features were fever in 19(63%) and headache in 26(87%)cases followed by altered sensorium in 12(40%) cases diplopia in 8(27%) cases and seizures in 3(10%) cases and the commonest examination findings were papilledema (n=23;77%) and meningismus (n=25; 83%). Neuroimaging was normal in 12(40%) cases showed tuberculomas in 11(37%) cases meningeal enhancement in 6(20%) cases basal exudates and hydrocephalus in 2 cases and vasculitic infarct in one case. Conclusions: In our study the clinical profile was similar to earlier studies from India however neuroimaging was normal in 40% patients. This study highlights that clinical features of tubercular meningitis are often non specific and classical features like meningeal enhancement basal exudates hydrocephalus may be absent on neuroimaging in approximately one third of cases. Diagnosis requires high index of suspicion and careful examination for signs of meningitis.
SawanVerma, Irfan Yousuf, Mushtaq Ahmad Wani, RavoufAsimi, MaqboolWani
Sher-e-Kashmir Institute of Medical Science, Jammu and Kashmir, India
Brucellosis is a common zoonotic infection in many parts of the world including the Mediterranean and Middle Eastern countries. The disease is caused by the bacterial genus Brucella. Brucellosis is transmitted to humans by direct contact with infected animals through cuts and abrasions or inhalation of aerosols or by ingestion of unpasteurized milk or milk products. Nervous system is affected in 5% both acute and chronic presentation are recognized with a broad spectrum of neurological manifestations. We are presenting few cases of Neurobrucellosis with different neurological manifestation.
Severe GeneralisedTetanus; A Case Report and Review of literature
Omar Farooq,Ishrat Hussain Dar, Drsamia Rashid
Government Medical College Srinagar, Jammu &Kashmir, India
Tetanus is a toxin mediated disease produced by the bacterium clostridium tetani. It is characterized by generalized rigidity and muscle spasms that may cause respiratory arrest and death. Tetanus typically gains access to the body through evident or inevidentwounds. Implementation of mandatory vaccination programmes has successfully decreased the prevalence of this disease world wide. We report a case of generalized tetanus in a male patient who presented to this hospital with inability to open mouth and severe stiffness of whole body with non healing ulcer of hand. The patient was admitted to the intensive care unit and was given the full supportive therapy for tetanus. The patient was discharged in good condition.
A Case Series of JC Virus Positivity
Nandakumar V., Lakshminarasimhan R., Muthukumaran S., Kannan. V, Thamilpavai N., Sarala G., K. Bhanu
Madras Medical College, Chennai, Tamilnadu,India
Introduction: The John Cunningham virus [JCV] is a type of human polyomavirus. Seroprevalence studies had demonstrated antibodies to JCV in 80 percent of adults but it remain asymptomatic in immunocompetent individuals. JCV is cytolytic to oligodendrocytes. JCV causes progressive multifocal leukoencephalopathy[PML]JC virus cerebellar granule cell neuronopathy JC virus meningitis only in immunosuppressed states. In blood an antibody titre index of >0.40 denotes anti-JCV antibody positivity. Aim: To identify the varied clinical profile of JC virus. Case vignette 1:51 year old maleadmitted with memory disturbances apathy urinary incontinence. Patient was HIV positive. MRI showed multifocal asymmetric periventricular and subcortical whitematter lesions. Antibody titre for JCV is 0.5. Case vignette 2:34 year old lady a case of relapsing remitting multiple sclerosis had frequent relapses even with interferon therapy was admitted with paraparesis. Natalizumab was started. MRI showed T2 hyperintense lesions in periventricular bilateral cerebral whitematter. Antibody titre for JCV is 0.6. Case vignette 3:36 year old lady admitted with cognitive decline dysphagia speech disturbances. MRI showed T2 hyperintense periventricular white matter lesions in bifrontal right peritrigonal region right cerebellum. Antibody titre for JCV is 0.6. Discussion: In our case series patient one is HIV with PMLimproved with HAART. Patient two is a case of RRMS treated with natalizumab shifted to dimethyl fumarate had no further relapses. Patient three is a case of primary JCV disease treated symptomatically but did not show significant improvement. Conclusion: In contrast to literature evidence our patient with HIV and PML improved dramatically to HAART. Optic nerve and spinal cord are not affected in our patients.
A Case of Locked in Syndrome due to Neurotoxic Snake Bite
Moses P Murthi,S. Balasubramanian, K. Bhanu
Madras Institute of Neurology, Erode, Tamil Nadu,India
Neurotoxic snake bite is a common emergency medical problem in the tropical countries. Neurological manifestationare mainly due to neuro muscular paralysis. Locked in syndrome is a condition in which the patient is not able to communicate eventhough they are conscious. Here we are reporting a rare case of complete locked in syndrome. In this case the patient was admitted with total quadriplegia bulbar paralysis respiratory failure with ophthalmoplegia with ptosis suggestive of complete locked in syndrome. The patient was treated in appropriate time with ventilatory support anti snake venom and parental neostigmine. Patient recovered well on 10th day without any residual neurological deficit. Electrophysiological evaluation done after recovery was normal. This case is presented for its rarity and for documentation.
Intracranial Tuberculoma A Therapeutic Failure
Jyoti Garg, Prashant Kumar, AnkurWadhwa, Abhishek Kumar, Kuljeet Singh Anand, ShahinaBano
PGIMER & Dr. RML Hospital, New Delhi, Delhi, India
Background: Intracranialtuberculomas are common problem in developing countries. The mainstay of treatment is ATT but there are no definitive guidelines for the doses number of antitubercular drugs duration of treatment and role of steroids. Though the literature says most of the tuberculomas resolve with 18-24 months of ATT we report a series of β€" non resolving IC tuberculomas even with much longer ATT duration. Objective β€" To ascertain the response of antituberculous treatment in patients of IC tuberculomas. Method: 42 consecutive patients of IC tuberculomas attending neurology OPD were included in the study. Diagnosis of IC tuberculoma was made on the basis of clinical and characteristic imaging finding. Detailed drug history including duration of use of steroid and response to treatment by serial clinical assessment and imaging at 6-8 months were recorded. Results: A total of 42 cases (age range 11-53 years M: F 13:29) of IC tuberculomas were studied. 17(40% M: F- 5:12) patients showed persistent non resolving contrast enhancing lesions in serial MR imaging even with 18-48 month of DOTS/regular ATT. Out of these 17 patients 10 showed no regression of lesions 5 had increase in size of lesions and 2 showed appearance of new lesions on serial imaging. Conclusion: Justification to stop and duration of ATT is a matter of debate and further research.
Clinical and Aetiological Profile of Acute Meningoencephalitis in Kashmir J & K - A Hospital Based Study.
Omar Farooq, Ishrat Hussain Dar, Mohsin Manzoor, Samia Rashid Mir, Javid Basu, Mohammed Ashraf
Government Medical College Srinagar, Srinagar, J & K,India
The present study was conducted in the Post Graduate Department of Medicine Neurology Division at SMHS Hospital Srinagar an associated hospital of Govt. Medical College Srinagar. This study was conducted from March 2013 to October 2014. Patients presenting with febrile illness with signs of meningeal irritation were included in this study. A total of 203 patients were studied in whom detailed history neurological examination and CSF analysis was done along with neuroimaging-CT Head/ MRI Brain. Patients were categorized into three groups: pyogenic meningitis tubercular meningitis and viral meningoencephalitis. Among those 41.9% were TBM 34.5% were viral meningoencephalitis and 23.6% were pyogenic meningitis. A triad of fever headache and signs of meningitis were present in 75% of pyogenic meningitis 49.41% in TBM and 22.8% in viral meningoencephalitis. The most common detected pyogenic causative organism was S. pneumoniae in 11.11% and viral causative organism was HSV 1 in 28.57% patients. Most of the TBM patients (67.10%) presented to hospital in MRC Stage II. Appropriate attention is needed for diagnosing and categorization of various meningitides which is beneficial for the outcome of patients.
Spectrum of Intra-Cranial Tuberculosis
Baiakmenlang Synmon, Ashok K. Kayal DM, Munindra Goswami, Moromi Das, Lakshya J Basumatary
Gauhati Medical College and Hospital, Guwahati, Assam, India
Central nervous system tuberculosis is the most severe extra pulmonary tuberculosis having a high mortality and morbidity. We aim to study the various clinical biochemical and radiological spectrum of intracranial tuberculosis. Materials and Method: Seventy patients were enrolled in this prospective study after ethical clearance and consent. Clinical evaluation routine blood & radiological investigation cerebrospinal fluid analysis was done to confirm tuberculosis and exclude other infections. The patients were graded with MRC scale managed followed up and outcome was noted with Glasgow outcome scale (GOS). Results: 25 females and 45 males were included whose mean age of presentation was 33.14 Β 17.5 years. Alcohol was the most common risk factor seen in 22.9%. History of contact and a past history of tuberculosis were found in 10% and 27% respectively. Headache (88.6%) was the most common symptom. Co-infection with HIV cryptococcal& toxoplasmosis were seen in 6 3 & 1 patient respectively. CSF analysis showed AFB in 1 patient PCR for TB and BACTEC was positive in 1 & 2 patients respectively. Neuroimaging showed basal exudates (21.7%) tuberculoma (28.6%) brain oedema (27%) hydrocephalus (32.9%) infarct (21%) and abscess (2.9%). Complication like brain oedema (31.4%) vasculitis (20%) hydrocephalus (18.6%) hyponatremia (11%) drug induced hepatitis (5.7%) and rash (1.4%) was noted. Twenty percent patient died and 37.1% developed neurological sequelae. Logistic regression showed that a Glasgow scale of <10 MRC stage 3 and vasculitis were associated with poor outcome. Conclusion: Lack of sensitive diagnostic method and criteria makes CNS tuberculosis a challenge where early diagnosis and management is required.
Opsoclonus Myoclonus Ataxia Syndrome in Scrub Typhus
Kalpana Radhakrishnan, Muthuraj, V. Nagarajan, S Sundar
SRM Medical College Hospital KattankulathurKanc, Erode, Tamilnadu, India
11 yr old male child was admitted in our hospital with complaints of fever head ache vomiting for the past 5 days and unsteadiness of walk for the past 2days O/E the child was febrile irritable neck stiffness was present. He was severely ataxic- truncal and limb. He also had opsoclonus and myoclonus. Intermittently the child had generalized myoclonic jerks. Careful examination revealed eschar behind his left ear. MRI brain imaging showed diffusion restricting lesion β€"acutedemyelenation involving the cerebellar peduncules and pons. CSF analysis showed mild lymphocytic pleocytosis. He was treated with azithromycin doxycycline on day 1. A parenteral steroid was given on day 1 additionally as the initial diagnosis considered was ADEM. However on the next day when the fever work up showed positive scrub typhus IgM antibody steroid was stopped. Treatment with azithromycin docxycyclin was continued and the child showed remarkable recovery over the next 2 days. Conclusion: This case is presented to highlight the occurrence of OMA syndrome in scrub typhus and the complete recovery of the same with treatment for scrub typhus. Reference1. Opsoclonus in scrub typhus post grad med 2012 D`Sa S Singh S Sowmya S.2. Scrub typhus encephalomyelitis with prominent focal neurologic signs. Kim DE Lee SH park KI3. Scrub typhus β€"associated acute disseminated encephalomyelitis. Pei-HaochenKuo- Hua Hung.
Broken Heart in Tubeculous Meningitis
Josehva Caleb K, Muralidhar Reddy Y, Nilesh Vijay Chaudary, Shyam K Jaiswal, Murthy JMK
Institute of Neurological Sciences Care Hospital, Hyderabad, Telangana,India
Introduction: Takotsubo cardiomyopathy (TCM) or broken heart has been described as complication commonly in subarachnoid hemorrhage and traumatic brain injury. To date there is description of this complication in CNS infection. This report describes this complication in a patient with tuberculous meningitis. Case: A 38-yr-old female presented with holocranial headache and episodes of vomiting of one week duration and altered sensorium since one day. Examination revealed a drowsy and arousable patient with meningeal signs and no lateralizing deficits. Cerebrospinal fluid (CSF) analysis showed CSF opening pressure of 30 cm of water; cell count: 110 cells/mm3 (97 % lymphocytes); proteins: 112.5 mg/dl glucose: 45 mg/dl ADA: 8 u/L and positive TB PCR. Brain imaging showed basal exudates and no parenchymal lesions and hydrocephalus. On day-3 of admission she developed breathlessness tachycardia and hypotension. Cardiac evaluation: ECG showed T wave inversions in lateral leads elevated troponin I dilated left ventricle with severe systolic dysfunction and apical hypokinesia (Takotsubo Cardiomyopathy) on 2D Echo. Serum cortisol was normal. She was diagnosed to have TCM and treated conservatively. Echocardiography and 2D Echo done on day-5 were normal. She is on anti-tuberculous drugs and being followed-up Conclusion: Our patients meets the clinical and echocardiographic criteria of TCM. This is probably the first documentation of TCM in tuberculous meningitis. We suggest that TCM should be suspected in patients with CNS infection with unexplained hypotension and breathlessness.
Poster Session 19, Saturday, October 03, 2015,
04: 00 PM - 06:00 PM
Dr. Vimla Vermani Hall (Hall D):
Mitochondrial Disorders due to Polymerase Gamma2 (POLG2) Mutations: A Phenotypic, Biochemical and Histopathological Study.
Shwetha Chiplunkar, Kothari Sonam, ParayilSankaran Bindu, Arun BTaly, Narayanappa Gayathri, Srinivas Bharath MM, MadhuNagappa, Rakesh kumar, SanjibSinha, Hanumanthapura R Arvinda
National Institute of Mental Health & Neurosciences, Bangalore, Bangalore, India
Background: Mitochondrial DNA replication and repair is a function of polymerase gamma (POLG). Syndromes due to mutations in the main catalytic sub-unit, POLG1, are common and well-characterized. Knowledge of disorders caused by mutations in the accessory sub-unit (POLG2) is sparse. Aim: To describe phenotypic, pathological, biochemical and imaging correlates of POLG2 mutations. Methods: Over a period of two years (2011-2013), 102 patients fulfilling the modified Walkers criteria for mitochondrial cytopathyunderwent sequencing of POLG2. Seventeen patients (age range: 2-58 years; M: F (2:1) showed mutations in POLG2. Results: Clinical phenotypes included proximal myopathy (n=9), chronic progressive external ophthalmoplegia (n=4), extra-pyramidal syndrome (n=2), epilepsy (n=1) and Leigh-like illness (n=1). Consanguinity was noted in three. Family history was present in five and clinical phenotype resembled the proband in three. Eight patients had normal brain MRI. Others showed dentate mineralization (n=2), diffuse atrophy (n=2), striatal necrosis (n=1), globuspallidushyperintensity (n=1) and temporal cyst (n=1). Muscle biopsy(n=16) showed cytochrome c oxidase (COX) deficientand ragged red fibres (n=4), isolated COX deficiency (n=1) and type II fibre atrophy (n=2). Spectrophotometric analysis of respiratory chain complexes (n=14) showed isolated deficiency of complex I (n=3) and complex IV (n=1). Five patients had multiple complexdeficiencies. Homozygousmutations were noted inR369G (n=14), D352N (n=1), Y349X(n=1) and D479N (n=1) of POLG2 gene. Conclusion: Mutations in POLG2 are associated with a wide range of clinical manifestations, the commonest being neuromuscular. POLG2 should be sequenced routinely in all patients with suspected mitochondrial cytopathy, particularly when POLG1 is normal.
Do Cluster Headache patients have Cognitive Dysfunctions?
Soumya Sharma, Debashish Chowdhury, Ruby Chopra, Geeta Khwaja, Sanjay Pandey
GB Pant Institute of Post graduate Medical Education and Research, New Delhi, Delhi, India
Background: Only a few studies of cognitive dysfunctions in cluster headache (CH) have been reported previously. Studying cognitive functions may facilitate better understanding of pathophysiologyofCH. Objective: To assess the occurrence impairment of attention, vigilance and executive functions in CH.
Methods: CH patients in cluster periods during headache free intervals were prospectively studied by a battery of tests including Frontal assessment battery (FAB), Digit span, Stroop color word interference test, Trail making test (TMT) A and B and P300 event related potentials (ERP).
Results: Forty two CH patients (30 ECH and 12 CCH) with mean age 38.3 years and male to female ratio of 2.2 were seen from August 2013 to May 2015. Average symptom duration was 4.59 years. 29 patients had formal education up to eighth standard or higher, six were illiterates. Therefore, all patients could not perform all the tests. FAB scores were impaired in 69% (n=29/42) patients. All CCH patients had impaired FAB. TMT-A was impaired in 17.9% (n=7/39) and TMT-B in 9.09% (n=3/33). Forward and backward digit span tests were impaired in all patients. In Stroop color interference test, 27/37 patients (72.9%) committed errors; their average time was significantly higher (77.8 seconds vs 56.8 seconds) than those who didn't commit errors. P300 ERPs done in 17/42 patients showed significant prolonged latencies (mean = 441.31ms) and lower mean amplitude (mean =19.22uV) compared to lab normative data. Conclusion: Cognitive dysfunctions are common in CH patients in cluster period during headache free intervals. Impairment of attention and vigilance is more common than executive dysfunctions.
Analysis of Neurological Soft Signs in Primary Headache Patients
Thamilpavai. N, Lakshminarasimhan. R, Bhanu. K
Introduction: NSS correlates with neuroanatomical, neurocognitive abnormalities which represents defective neural intregration. Neurological examination is usually normal and investigations are non contributory in patients with primary headaches. NSS can be used to identify a specific subset of headachepatients. Aim of Study: To assess neurological soft signs in primary headaches using Heidelberg scale. Materials and Methods: It is a crosssectional study of 60patients attending our headache and neurological OPD in RGGH Chennai. Diagnosisof headache made by HIS criteria. Inclusion Criteria: Age >18 and < 60 years. H/O of > 1 to < 15 attacks / month. Exclusion Criteria: H/O intake of psychoactive medications,psychiatric illness, head trauma, secondary headaches. Detailed neurological examination and brain imaging done. Results: Of the 60 patients examined 55 of them presented with migraine, 5 with tension type headache with a mean duration of 1 - 10 years. The NSS score was increased in patients with a longer duration of headache. 20 of the 60 had increased scores .6 of the 20 had increased scores in all the domains.7 of them had defective motor coordination,3 in spatial orientation, 4 in complex motor tasks. Only 5 patients had WMH lesions. Conclusion: Patients with chronicisation of headaches had increased NSS scores. Endophenotypes of psychiatric comorbitidies may be present which may be missed by routine clinical examination. Tailored pharmacotherapy may be beneficial and cost effective.
Perfusion MRI Predicts Drug Response in Brain Tuberculoma
Roase Dawn Bharath, Gudipati Anantaram, S R Chandra, Jitender Saini, A K Gupta
NIMHANS, Bangalore, Karnataka, India
MRI is considered gold standard imaging investigation in the diagnosis of central nervous system tuberculosis and often guides the initiation of Anti tuberculous therapy (ATT). We aimed at predicting the response to drug by evaluating the rCBV values in patients with brain tuberculomas (BT). Nineteen patients in the age range of 4-50 years proven to have BT were serially evaluated using T2* Perfusion on 3 Tesla MRI system before starting ATT. All patients were treated using the same protocol for 12-22 months. At the end of therapy patients were classified into two groups based on clinical and imaging response: responders (n = 13) included those patients who showed clinical and imaging improvement. Non-responders (n = 6) included patients who did not show clinical/ imaging response. We found the rCBV of the peripheral wall of lesions in responders varied from 0.87 to 3.5 (mean SD: 1.57 0.66). The rCBV of peripheral wall of the lesions in non-responders varied from 0.15 to 1.44 (mean SD: 0.66 0.51). The lesions in the responders had significantly higher rCBV ratios in the center and periphery of lesions than those in the Non-responders (p < 0.05). We conclude that presence of very low rCBV values on initial study in a tuberculoma could predict nonresponse to ATT
Cytokines and matrix metalloproteinases in the cerebrospinal fluid of patients with acute transverse myelitis: an outcome analysis
Hardeep S. Malhotra, Puneet Dixit, Ravindra Kumar Garg, Amita Jain, Rajesh Verma, Praveen Kumar Sharma, Neeraj Kumar
King George Medical University, Lucknow, UP, India
Background: Role of cytokines as well as matrix metalloproteinases (MMPs) is well defined in various central nervous system inflammatory diseases but data regarding their role in acute transverse myelitis is limited. Material and Method: In this study patients with acute transverse myelitis, fulfilling the inclusion and exclusion criteria defined by Transverse Myelitis Consortium Working Group, were enrolled. Study also included controls. Cerebrospinal fluid (CSF) samples both from patients and controls were obtained. All CSF samples were analysed for cytokines (interleukin (IL)-6, IL-8, IL-10 and IL-17) and matrix metalloproteinases (MMP-2, MMP-9). A detailed clinical evaluation and magnetic resonance imaging were done at inclusion. Patients were followed up for 3 months. Disability was assessed using modified Barthel index (MBI). Results: CSF levels of cytokines IL-6, IL-8, IL-10, MMP-2 and MMP-9 were significantly higher in patients with transverse myelitis as compared to the controls (p<0.001). No significant difference in the levels of IL-17 was noted. CSF IL-6 and IL-8 were significantly associated with severity of the disease (MBI≤12). After 3 months, presence of quadriparesis (p=0.001, odd's ratio=19.5, 95% CI 2.34-62.39) and long segment myelitis (p=0.035, odd's ratio=4.37, 95% CI 1.17-5.95) were significantly associated with poor outcome. Amongst the estimated CSF cytokines and matrix metalloproteinases, only IL-6 levels at baseline correlated significantly with the MBI at 3 months (spearmen's rho= -0.335, p=0.01). Conclusion: Anti-inflammatory and proinflammatory cytokines, MMP-2 and MMP-9 were elevated in acute phase of transverse myelitis. Higher CSF IL-6 levels at baseline were associated with more disability (MBI≤12) at 3 months.
To study the frequency of known primary mutations in patients of Leber's Hereditary Optic Neuropathy from North India
Pulikottil Wilson Synmon, Radhika P Ramachandran, B Ramya, Modi M, Gupta A, Lal V
PGIMER, Chandigarh, India
Objective: To study the frequency of known primary mutations in patients of Leber's Hereditary Optic Neuropathy from North India. Background: The mutations G11778A, T14484C, and G3460A contribute to >95% of LHON in populations of European decent. However, the relative frequency of each of these pathogenic mutations varies considerably worldwide. The predominance of G11778A is higher in East Asia than in European derived populations. In this study we assessed the frequency of known primary LHON mutations in patients presenting with Leber's Hereditary Optic Neuropathy. Methods: Complete neurological and ophthalmological examinations were carried out accompanied by inflammatory and infective blood markers. Neuroimaging was done in all cases to exclude focal lesions. Fundus flourescein angiography was performed in acute cases. Blood samples were drawn and tested for primary mutations through PCR amplification followed by confirmation using Sanger technique. Results: Four patients tested positive for primary LHON mutations. Two patients were homoplasmic for 14484 mutations and two for 11778 mutations. None of the tested patient carried the 3460 mutation. None of the control population tested positive for any of the three known primary LHON mutations (P=0.029). Conclusion: Our study shows that detecting the primary LHON mutations namely G11778A, T14484C, and G3460A in patients from North Indian population definitively diagnoses Leber's Hereditary Optic Neuropathy. However sensitivity of this test is substantially lower in Indian population when compared to the Western population. Also the high cost involved in doing this test does not justify its use as a screening tool in the evaluation of LHON.
Refractory Psychosis as First Manifestation of Neuropsychiatric Lupus
S. R. Chandra, Thomas Gregor Issac, Krishnan Ayappan, Anita Mahadevan
NIMHANS, Bangalore, India
Introduction: First episode of psychosis can occur at any age and it can be primarily psychiatric or secondary to other occult diseases. It is of great therapeutic relevance to be cautious about organic etiology as early diagnosis can help in early initiation of disease modifying treatments. Aim: To study patients who presented with first episode of psychosis and later turned out to be due to lupus erythematosus with varying periods of delay. Study Design: Details of patients who were considered as treatment unresponsive psychosis and later turned out to be due to vasculitis were entered in excel sheet and analyzed. Patients and Methods: The details of patients including neuropsychological features, apparent soft signs which were initially ignored, lab data and signs during follow up were tabulated and warning signs analyzed. Results: All our patients were highly intelligent young females and there were stressors in all of them. They presented with panic, followed by psychotic symptoms which was resistant to regular psychiatric treatment and therefore possibility of organicity explored. All of them had history of transient symptoms referable to other systems which were treated as such and patients did not volunteer the same unless questioned. During follow up full-fledged features of SLE came up in all patients. Conclusion: When a young female with no past or family history of mental illness presents with psychotic features, unprovoked panic, and limbic symptoms always ask for minor or transient symptoms and signs referable to the other systems which might give valuable clues.
Factors predicting seizure outcome following resective surgery in malformations of cortical development
Deepak Menon, Hardeep Kumar, Ashalatha Radhakrishnan, Ramsekhar Menon, Mathew Abraham, George Vilanilam, Bejoy Thomas, C. Kesavadas, Ravi Varma Prasad, Sanjeev V. Thomas
SCTIMST, Trivandrum, Kerala, India
Introduction: Malformations of cortical development(MCD) encompasses heterogenous entities which are major causes of drug refractory epilepsies among both pediatric and adult population. In this study, we reviewed clinical, neuropsychological, imaging, electrophysiological, pathological and seizure outcome of a large cohort of patients with MCDs who underwent presurgical evaluation through our comprehensive epilepsy care program. Objective: To determine factors predicting seizure outcome following resective surgery in MCD and to analyse various factors which caused surgical deferral in these patients. Methods: Consecutive patients with MCD who presented for presugical evaluation were selected. MCD was diagnosed depending on characteristic MRI findings and confirmation by histopathology. We analyzed clinical characteristics, preoperative MRI findings, electrophysiological features, electrocorticographic, pathological features and seizure outcome, at last follow-up/at end of two years, comparing the seizure free and seizure recurrence groups. Results: 148 patients with MCD were identified; 69(46%) underwent respective surgery. Multifocal epileptogenicity(44%), proximity to eloquent area(27%), and infrequent seizures/opting out(29%) were the reasons for deferral. 23(33.3%) had a relapse of seizures postoperatively. Univariate analysis showed completeness of resection (p=0.02) and abnormal post-operative EEG (p=0.01) influenced seizure outcome. On multivariate logistic regression, independent predictors of seizure freedom were shorter duration of epilepsy (OR 1.19, 95% CI 1.02-1.39, p=0.02), completeness of resection (OR 8.2; 95% CI 1.43-64.96, p=0.01), and absence of spikes in 1year post-operative EEG (OR 4.2; 95% CI 2.52-16.6; p<0.002). Conclusion: Completeness of resection, abnormal EEG at the seventh day of surgery and short duration of epilepsy were identified as significant predictors of seizure outcome.
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