Annals of Indian Academy of Neurology
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   2012| January-March  | Volume 15 | Issue 1  
    Online since February 29, 2012

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Paraneoplastic neurologic syndrome: A practical approach
Sudheeran Kannoth
January-March 2012, 15(1):6-12
DOI:10.4103/0972-2327.93267  PMID:22412264
Paraneoplastic neurological syndromes (PNS) are rare disorders associated with cancer, not caused by direct invasion, metastasis or consequences of treatment. They are usually autoimmune in nature. Often, PNS precedes the manifestations of cancer. Onconeural antibodies are important in the diagnosis and management of these disorders. These antibodies are specific for the malignancy rather than for a particular neurological syndrome. Often, there are different antibodies associated with the same syndrome. Multiple antibodies are also known to coexist in a given patient with malignancy. While investigating a patient for suspected PNS, the entire gamut of onconeural antibodies should be investigated so as not to miss the diagnosis. In 30-40% of the cases, PNS can occur without antibodies. Investigations for identifying the underlying cancer can be directed by the antibody panel. If conventional screening for cancer is negative, a positron emission scanning/computed tomography scan can be useful. Patients need follow-up surveillance for cancer if not detected in the first instance. Cancer detection and treatment, immunotherapy and supportive care are important components of treatment of PNS. Immunotherapy is very effective in PNS associated with cell membrane-associated antibodies like voltage-gated potassium channel complex, NMDA receptor antibodies and voltage-gated calcium channel antibodies. Immunotherapy includes steroids, IVIgG, plasmaphereis, cytotoxic medications and rituximab. Supportive therapy includes symptomatic treatment with antiepileptic and analgesic medications, physiotherapy, speech therapy and occupational therapy. PNS can mimic any neurologic syndrome. A high index of clinical suspicion is important for early diagnosis and prompt management and better outcome.
  11,243 568 27
Low cholesterol as a risk factor for primary intracerebral hemorrhage: A case-control study
Ashraf V Valappil, Nilesh V Chaudhary, R Praveenkumar, Biju Gopalakrishnan, AS Girija
January-March 2012, 15(1):19-22
DOI:10.4103/0972-2327.93270  PMID:22412267
Introduction: An inverse association between serum cholesterol and the risk of hemorrhagic stroke has been noted in epidemiological studies. We performed a case-control study to assess the relationship between primary intracerebral hemorrhage (ICH) and low serum cholesterol. Materials and Methods: Prospectively recruited fully evaluated patients with ICH were compared with a control group based in a primary care practice, i.e. age- and sex-matched individuals attending the routine preventive health check-up. Low cholesterol was defined by the sex-specific lowest quintile of the population. Results: The proportion of ICH patients with low cholesterol was significantly higher than the controls (68% vs. 43%). Mean total cholesterol was also signficantly low in ICH patients compared with controls (177 mg/dL vs. 200 mg/dl; P-value = 0.0006). Low-density lipoprotein cholesterol (LDL-c) and triglycerides were also significantly low in ICH patients compared with controls. Mean LDL-C in the ICH patient group was 114 mg/dL, whereas it was 128.5 mg/dL in the control group (P-value = 0.016). There was no significant difference in the high-density lipoprotein (HDL) levels in both groups. In a subgroup analysis, both men and women in the ICH group had a significantly low mean cholesterol compared with the control group. Although lower mean cholesterol was seen in both young and older individuals in the ICH group than in controls, the difference was significant only in the older group (age >45 years). In multivariate analysis, presence of low cholesterol remained a significant predictor of hemorrhage. The odds ratio of low cholesterol in the hemorrhage cases was 2.75 (95% CI = 1.44-5.49) unadjusted and 2.15 (1.13-4.70) adjusted for age and hypertension. Conclusions: This study confirms an increased risk of primary ICH associated with low cholesterol both in men and women, especially in older individuals.
  8,822 225 7
Mycobacterium avium-intracellulare brain abscess in HIV-positive patient
Sampada S Karne, Shashikala A Sangle, Dilip S Kiyawat, Sujata N Dharmashale, Dilip B Kadam, Renu S Bhardwaj
January-March 2012, 15(1):54-55
DOI:10.4103/0972-2327.93282  PMID:22412276
Mycobacterial opportunistic infections are a major cause of morbidity and mortality among patients living with HIV (PLHIV) worldwide. Nontuberculous mycobacterial (NTM) infection is one of the leading causes of opportunistic infection in patients with advanced acquired immunodeficiency syndrome i.e., with CD4 count less than 50/ Mycobacterium avium complex (MAC) is among the most common opportunistic bacterial infections in those patients with advanced immunodeficiency apart from cryptococcal meningitis, progressive multifocal leukoencephalopathy, etc. Common presentations of mycobacterium avium complex are fever, lymphadenitis and respiratory disease. Immune reconstitution disease is also known to manifest with MAC infections in PLHIV on highly active antiretroviral therapy. Very few cases of central nervous system involvement due to NTM infection have been described. We are reporting a case of advanced acquired immunodeficiency who presented with brain abscess due to Mycobacterium avium intracellulare.
  6,710 103 9
Glutaric aciduria type I: A treatable neurometabolic disorder
Mahesh Kamate, Vishwanath Patil, Vivek Chetal, Pavan Darak, Virupaxi Hattiholi
January-March 2012, 15(1):31-34
DOI:10.4103/0972-2327.93273  PMID:22412270
Background and Objectives: Glutaric aciduria Type-I (GA-I) has characteristic clinical and neuroimaging features, which clinches the diagnosis in a majority of patients. However, there have been few case reports on GA-I from India. This study was undertaken to study the clinical presentations, metabolic profile, neuroimaging findings and outcome of patients with GA-I. Study Design: The present study was a retrospective study. Materials and Methods: Retrospective review of charts of patients with a diagnosis of GA-I was carried out from March 2008 to April 2010. The clinical, laboratory and neuroimaging findings were extracted in a predesigned proforma and the data was analyzed. Results: Eleven cases were found to have GA-1. Clinical presentation was quite varied. Follow-up of patients revealed that one patient with macrocephaly as the only clinical finding was developmentally normal. One patient with encephalitis-like illness steadily improved and started walking at 2 years. Two patients were bed ridden and had severe dystonia. One patient died during follow-up. The remaining six patients had dystonia and other abnormal movements, but had attained sitting without support and were not ambulatory. Conclusion: GA-I is not an uncommon disorder and diagnosis can be made easily based on clinical, laboratory investigations and neuroimaging findings. It is one of the treatable metabolic disorders and, if managed appropriately, favorable prognosis can be given.
  6,018 172 7
Restless leg syndrome, periodic limb movements, febrile seizures and Attention deficit hyperactivity disorder in an Indian family
Meena Gupta, Amit Batra, Anurag Trivedi, Debashish Chowdhury, Geeta A Khwaja
January-March 2012, 15(1):39-43
DOI:10.4103/0972-2327.93277  PMID:22412272
Restless leg syndrome (RLS) is a common neurological disorder which can affect individuals of all age groups and incidence increasing with age. It can cause severe sleep disruption and negatively impact quality of life of an individual. Its diagnosis is clinical, based on essential criteria of International RLS Study Group. It can be idiopathic or associated with various medical and other neurological disorders. Idiopathic RLS can be sporadic or may have a familial inheritance, with several genetic loci been reported till date. RLS has a strong association with periodic limb movements, both sleep and awake. Very few studies of familial RLS/Periodic limb movements in sleep and their associations have been reported. We report an Indian family with autosomal dominant RLS/PLMS, with RLS and PLMS as well as psychiatric disorders, febrile seizures and Attention Deficit Hyperactivity Disorder in different family members, over three generations.
  5,678 108 1
Rare association of central pontine myelinolysis with infantile tremor syndrome
Kalpana Datta, Supratim Datta, Indranil Dutta
January-March 2012, 15(1):48-50
DOI:10.4103/0972-2327.93279  PMID:22412274
Central pontine myelinolysis (CPM) is an acute demyelination within the central basis pontis. Though exact mechanism is not known it is seen commonly with rapid correction of hyponatremia and also with pontine ischemia or infarction, demyelinating diseases, pontine neoplasm and different metabolic diseases. We report a rare association of CPM in a patient of Infantile Tremor Syndrom (ITS). ITS is a syndrome of tremor, mental and physical retardation, pigmentary changes of hair and skin and anemia in malnourished children. Though first reported in Indian subcontinent many identical cases were reported from around the world. Our case is a 15 month old child with generalized tremor, mild hepatosplenomegaly with features of grade II malnutrition including skin and hair changes. All the signs and symtoms of tremor improved after treatment with the World Health Organization (WHO) protocol for protein energy malnutrition (PEM) and administration of propranolol without any side effects.
  5,129 124 3
Forty-year-old diabetic lady with tightness in lower limbs
Snehaldatta S Khade, Shrikant S Deshmukh, Pramod V Dhonde, Satish V Khadilkar
January-March 2012, 15(1):15-18
DOI:10.4103/0972-2327.93269  PMID:22412266
  5,066 162 -
Familial Guillain-Barré syndrome: First Indian report
Karkal Ravishankar Naik, Aralikatte Onkarappa Saroja, Basangouda P Patil
January-March 2012, 15(1):44-47
DOI:10.4103/0972-2327.93278  PMID:22412273
Guillain-Barré syndrome (GBS) is the commonest acute immune-mediated peripheral neuropathy. Specific human leukocyte antigen types have been found in patients with axonal and demyelinating subtypes of GBS suggesting genetic susceptibility in the generation of GBS. However, familial occurrence of GBS is rare and 42 patients from 20 families have been reported. Majority of them are from European countries and two families have been documented from Asian countries, while none have been reported from India. Electrophysiological characterization in familial GBS has been limited. We report the clinical and detailed electrophysiological findings in two affected brothers with familial GBS from India who had GBS five years apart. Both of them had mixed axonal and demyelinating features in nerve conductions and had complete clinical recovery. Our report documents the first Indian familial occurrence of GBS. Detailed genetic and epidemiological studies are required to find the true prevalence of familial GBS.
  5,021 136 6
Use of thrombolytic therapy in cerebral venous sinus thrombosis with ulcerative colitis
Kavitha Kothur, Subhash Kaul, S Rammurthi, V. C. S. Srinivasarao Bandaru, Suvarna Alladi T Suryaprabha, K Rukmini Mrudula
January-March 2012, 15(1):35-38
DOI:10.4103/0972-2327.93276  PMID:22412271
Cerebral venous thrombosis developing concurrently with active ulcerative colitis poses a therapeutic dilemma. We report the case of a 31-year-old woman who developed dural venous sinus thrombosis during the course of active ulcerative colitis in whom we accomplished clot lysis using intrasinus urokinase. The success of the procedure was assessed by improvement in the patient's neurological condition and resolution of imaging features without any bleeding complications. We also reviewed literature on various modalities of treatment of sinus venous thrombosis in patients with ulcerative colitis and outcome.
  5,046 109 3
Prevalence of mental retardation among children in RS Pura town of Jammu and Kashmir
Sunil Kumar Raina, Sushil Razdan, Renu Nanda
January-March 2012, 15(1):23-26
DOI:10.4103/0972-2327.93271  PMID:22412268
Objective: To determine the Prevalence of mental retardation in children 3 to 10 years of age. Materials and Methods: The study was conducted in the framework of a population based, single centre, cross-sectional study at R.S.Pura town, 22 kms south- west of Jammu city. Results: A total of 61 (0.79 percent) of the 7,707 children surveyed had positive screening results on the Ten Questions instrument. 56 (0.72percent) children were diagnosed as suffering from mental retardation. Serious mental retardation was diagnosed in 48 children and mild mental retardation was diagnosed in 8 children. The combined prevalence estimates of mild and serious mental retardation were 7.2/1000. No notable sex differences were observed for either serious or mild retardation. Interpretation: The prevalence rates of mental retardation among children less than 19 years of age in R. S. town compares favorably with studies from developed world.
  4,965 151 -
Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease - A case report and brief review of literature
Soumya Patra, Radheshyam Purkait, Tapankumar Sinhamahapatra, Abhijit Misra
January-March 2012, 15(1):60-62
DOI:10.4103/0972-2327.93285  PMID:22412278
Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.
  4,969 99 5
Hypertrophic pachymeningitis in a patient with Takayasu arteritis: One more association?
Pandurang R Wattamwar, Suyog A Doshi, Bejoy Thomas, Muralidharan Nair, Abraham Kuruvilla
January-March 2012, 15(1):56-59
DOI:10.4103/0972-2327.93284  PMID:22412277
Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory disease of the dura mater, described in association with various infections, systemic vasculitides such as Wegener's granulomatosis and giant cell arteritis. However, HP in association with Takayasu arteritis (TA) has not been described. We report a young woman who presented with headache, seizures, and right third and fourth cranial neuropathy. Magnetic resonance imaging of the brain showed HP in bifrontal and right temporal region extending to cavernous sinus. She was also found to have systemic hypertension, stenosis of left subclavian, and left renal artery with narrowing of abdominal aorta, satisfying the diagnostic criteria for TA. A detailed evaluation for secondary causes of HP failed to reveal an alternative etiology. This report describes an unusual association of HP in a patient with TA, also emphasizing that seizures and cranial neuropathy may further expand the spectrum of neurological manifestations in patients with TA.
  4,700 107 6
Mirror movements
Nilesh A Nadkarni, Shrikant S Deshmukh
January-March 2012, 15(1):13-14
DOI:10.4103/0972-2327.93268  PMID:22412266
  4,310 327 3
Temporal arteritis: A case series from south India and an update of the Indian scenario
Thomas Mathew, Sushanth Aroor, Anup J Devasia, Anita Mahadevan, Vineeta Shobha, Raghunandan Nadig, Raji Varghese, SK Shankar, G. R. K. Sarma
January-March 2012, 15(1):27-30
DOI:10.4103/0972-2327.93272  PMID:22412269
Objective: To study the clinical, pathological and prognostic profile of patients with temporal arteritis in India. Materials and Methods: The study was conducted in a tertiary care center from south India from 2005 to 2010 in the departments of neurology and medicine. The details of all patients that satisfied the ACR 1990 criteria for diagnosis of temporal arteritis were reviewed. The clinical presentation, laboratory parameters and biopsy findings of the patients were analyzed and compared with other studies from India done over the last decade. Results: A total of 15 patients were diagnosed with temporal arteritis. The male:female ratio was 1.5:1. The mean age of onset was 67.58 years. Mean time for detection after onset of symptoms was 2.56 months. Typical manifestations included headache (100%), temporal artery tenderness (100%), jaw claudication (20%), polymyalgia rheumatica (53%) and visual manifestations (20%). The erythrocyte sedimentation rate was elevated in all patients. Biopsy was done in 13 patients, with 11 of them being positive. All patients responded to steroids well, with most patients being symptom-free within the first 48 h of treatment. Conclusions: Temporal arteritis seems to be underdiagnosed in India, with all patients previously misdiagnosed, and with a mean time from symptom onset to diagnosis of 2.5 months. The clinical presentation of temporal arteritis in India appears to be similar to that of the West, with no gender preference and a slightly younger age group.
  4,352 177 3
Seizure, spinal schwannoma, peripheral neuropathy and pulmonary stenosis - A rare combination in a patient of Neurofibromatosis 1
Avas Chandra Ray, Nabendu Karjyi, Achintya Narayan Roy, Arup Kumar Dutta, Atanu Biswas
January-March 2012, 15(1):51-53
DOI:10.4103/0972-2327.93281  PMID:22412275
Neurofibromatosis 1 (NF1) is the most common neurocutaneous syndrome. It is estimated to occur in approximately 1 out of every 3300 infants. The manifestations of this condition are diverse and can arise from almost any system in the body. The neurofibroma is the hallmark lesion of NF1 that develops from peripheral nerves. Here, we are reporting an 18-year-old girl with NF1. Clinical diagnosis was made according to the diagnostic criteria established by the National Institutes of Health Consensus Development Conference in 1987. She presented with quadriparesis due to dumbbell-shaped spinal schwannoma in the cervical region. She had history of recurrent seizures in the past, with poor scholastic performance. There were clinical and electrophysiological features of peripheral neuropathy and clinical and echocardiographical features of pulmonary stenosis. These are uncommon features of NF 1. The presence of all these features in a single patient makes it a unique case.
  4,043 100 -
Chronic cerebrospinal venous insufficiency in Multiple Sclerosis: A note for caution
Rohit Bhatia, Abhishek , Kameshwar Prasad
January-March 2012, 15(1):2-5
DOI:10.4103/0972-2327.93266  PMID:22412263
  3,924 157 -
Eminent Neuroscientists: Their Lives and Works
Nadir E Bharucha
January-March 2012, 15(1):64-65
  3,654 96 -
Change of guard
Satish V Khadilkar
January-March 2012, 15(1):1-1
DOI:10.4103/0972-2327.93265  PMID:22412262
  3,075 114 -
The withering shine of white coat
Ramesh Aggarwal
January-March 2012, 15(1):63-63
DOI:10.4103/0972-2327.93286  PMID:22412279
  2,797 105 3
Neurologists' role in antimicrobial resistance
Hamidreza Mahboobi, Forough Mahmoudi, Mohammad Esmaeil Shahrzad, Tahereh Khorgoei
January-March 2012, 15(1):68-68
DOI:10.4103/0972-2327.93290  PMID:22412280
  2,448 91 -
Arun Shah
January-March 2012, 15(1):67-67
  2,434 92 -
Cysticercosis and ptosis
Somsri Wiwanitkit, Viroj Wiwanitkit
January-March 2012, 15(1):68-68
DOI:10.4103/0972-2327.93291  PMID:22412281
  2,318 65 -
Diagnosis and Management of Neurological Disorders
JA Lalkaka
January-March 2012, 15(1):66-66
  2,011 87 -
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