Annals of Indian Academy of Neurology
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   2014| January-March  | Volume 17 | Issue 1  
    Online since March 12, 2014

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Efficacy and safety of methylcobalamin, alpha lipoic acid and pregabalin combination versus pregabalin monotherapy in improving pain and nerve conduction velocity in type 2 diabetes associated impaired peripheral neuropathic condition. [MAINTAIN]: Results of a pilot study
D Vasudevan, Manoj M Naik, Qayum I Mukaddam
January-March 2014, 17(1):19-24
DOI:10.4103/0972-2327.128535  PMID:24753654
Background and Objective: To assess whether methylcobalamin and alpha lipoic acid (ALA) added to pregabalin provide additional benefit compared to pregabalin alone in type 2 diabetes mellitus associated peripheral neuropathy. Setting and Design: An open label, randomized, controlled parallel-group pilot study. Materials and Methods: Thirty adult patients with type 2 diabetes mellitus with symptoms of peripheral neuropathy for ≥6 months were randomized to receive pregabalin 75 mg, methylcobalamin 750 μg, and ALA 100 mg (PMA, n = 15); or pregabalin 75 mg (PG, n = 15) for 12 weeks. Assessment variables were numeric rating scale (NRS), sleep interference scores (SIS), response rate to pain, and global assessment for the usefulness of therapy. The nerve conduction velocity was assessed for sensory and motor nerves. Safety assessment included adverse events reported by the patients, clinical laboratory, and general medical, neurological examinations. Statistical Analysis: Efficacy analyses were done on per-protocol (PP) population, whereas safety analyses were done on intent-to-treat (ITT) population. Results: Significant improvement was seen in pain and sleep interference in both groups. Mean nerve conduction velocity of left common peroneal nerve (CPN) showed significant improvement in PMA group at week 12 compared to baseline (P = 0.018). For right CPN both groups showed significant improvement. (PMA, P = 0.002, PG, P = 0.007). For sensory testing, at week 12, right superficial peroneal nerve showed reduction in nerve conduction velocity in PG group compared to baseline (P = 0.043). Conclusion: Methylcobalamine, ALA and pregabalin combination provides pain relief and improves sleep interference. Addition of methylcobalamin and ALA to pregabalin improves the nerve function. Due to small sample size, most of the efficacy parameters could not reach significant difference between groups; hence benefit of the 3-drug-combimation should be interpreted with reservation.
  11,117 335 3
Isolated primary craniosynostosis in an adult: Imaging findings of a case
Shruti Thakur, Anupam Jhobta, Suresh Kumar, Charu Smita Thakur
January-March 2014, 17(1):92-94
DOI:10.4103/0972-2327.128563  PMID:24753669
Craniosynostosis means premature closure of calvarial sutures. It may be primary or secondary. The patient presents with unexplained neuropsychological impairment and radiological imaging clinches the diagnosis. We present a case of 31-year-old female having primary isolated craniosynostosis who survived into adulthood without any surgical intervention. The imaging findings of such a case are rarely described in the literature.
  9,414 57 -
Acute confusional state/delirium: An etiological and prognostic evaluation
Dheeraj Rai, Ravindra Kumar Garg, Hardeep Singh Malhotra, Rajesh Verma, Amita Jain, Sarvada Chandra Tiwari, Maneesh Kumar Singh
January-March 2014, 17(1):30-34
DOI:10.4103/0972-2327.128541  PMID:24753656
Introduction: Acute confusional state/delirium is a frequent cause of hospital admission, in the elderly. It is characterized by an acute fluctuating impairment of cognitive functions and inattention. Recognition and prompt treatment is crucial to decrease the morbidity and mortality associated with it. Materials and Methods: In this retrospective study, we determined the etiology and prognostic factors of an acute confusional state. A total of 52 patients of acute confusional state were clinically evaluated. All patients were also subjected to a battery blood biochemical examination, cerebrospinal fluid analysis and neuroimaging. Disability was assessed by using modified Barthel index (MBI). Patients were followed-up for 3 months. Results: The mean age of our cohort was 65.04 ± 10.6 years. 32 (61.5%) patients were male. In 33 patients, we were able to identify possible precipitating cause of an acute confusional state. In the rest of the patients results of all the tests were normal. Leukocytosis and hyponatremia were frequent factors associated with delirium. The mean duration of the hospital stay was 10.73 ± 3.6 days (range 5-21 days). Patients with an abnormal work-up (possible precipitating cause) had significantly lower mortality, less duration of hospital stay and less severe disability after 3 months. Age, underlying illness, serum creatinine, abnormal neuroimaging and MBI were identified as a significant prognostic indicator. 18 (34.6%) of our patients died, of these in 10 patients we could not find a precipitating cause. Conclusion: Patients, in whom a cause was found out, had better prognosis in terms of lesser mortality and the duration of hospital stay.
  5,728 165 -
Intractable vomiting and hiccups as the presenting symptom of neuromyelitis optica
Girish Baburao Kulkarni, Pradeep Kallollimath, R Subasree, M Veerendrakumar
January-March 2014, 17(1):117-119
DOI:10.4103/0972-2327.128575  PMID:24753677
Vomiting and hiccups can be due to peripheral or central causes. Neurological diseases causing vomiting and hiccups are due to lesions of medulla involving area postrema and nucleus tractus solitarius. Neuromyelitis optica (NMO) is one such disease which involves these structures. However refractory vomiting and hiccups as the presenting symptom of NMO is unusual. Here we report a patient with NMO in whom refractory vomiting and hiccups were the sole manifestation of the first attack. Diagnosis can be missed at this stage leading to delay in treatment and further complications. This case demonstrates the importance of considering NMO in any patient presenting with refractory vomiting and hiccups and with local and metabolic causes ruled out and linear medullary lesion on magnetic resonance imaging may indicate the diagnosis even when the classical clinical criteria are not met. Anti NMO antibody testing should be done and if positive appropriate treatment should be initiated to prevent further neurological damage.
  5,448 81 -
Susceptibility weighted magnetic resonance imaging of brain: A multifaceted powerful sequence that adds to understanding of acute stroke
Deepti Naik, Sanjaya Viswamitra, Ashok A Kumar, MG Srinath
January-March 2014, 17(1):58-61
DOI:10.4103/0972-2327.128555  PMID:24753661
Context: To evaluate the additional information that susceptibility weighted sequences and datasets would provide in acute stroke. Aims: The aim of this study were to assess the value addition of susceptibility weighted magnetic resonance imaging (SWI) of brain in patients with acute arterial infarct. Materials and Methods: All patients referred for a complete brain magnetic resonance imaging (MRI) between March 2010 and March 2011 at our institution had SWI as part of routine MRI (T1, T2, and diffusion imaging). Retrospective study of 62 consecutive patients with acute arterial infarct was evaluated for the presence of macroscopic hemorrhage, petechial micro-bleeds, dark middle cerebral artery (MCA) sign and prominent vessels in the vicinity of infarct. Results: SWI was found to detect hemorrhage not seen on other routine MRI sequences in 22 patients. Out of 62 patients, 17 (10 petechial) had hemorrhage less than 50% and 5 patients had greater than 50% area of hemorrhage. A "dark artery sign" due to thrombus within the artery was seen in 8 out of 62 patients. Prominent cortical and intraparenchymal veins were seen in 14 out of 62 patients. Conclusions: SWI has been previously shown to be sensitive in detecting hemorrhage; however is not routinely used in stroke evaluation. Our study shows that SWI, by virtue of identifying unsuspected hemorrhage, central occluded vessel, and venous congestion is additive in value to the routine MR exam and should be part of a routine MR brain in patients suspected of having an acute infarct.
  5,097 99 1
Hyponatremia in stroke
Sheikh Saleem, Irfan Yousuf, Azhara Gul, Satish Gupta, Sawan Verma
January-March 2014, 17(1):55-57
DOI:10.4103/0972-2327.128554  PMID:24753660
Introduction: Hyponatremia is a common electrolyte disorder encountered in patients of neurological disorders which is usually either due to inappropriate secretion of Antidiuretic hormone (SIADH) or cerebral salt wasting syndrome (CSWS). We conducted this study in a tertiary care hospital to determine the incidence and etiology of hyponatremia in patients of stroke admitted in the hospital. Materials and Methods: It was a prospective study done over a period of two years that included established cases of stroke diagnosed on the basis of clinical history, examination and neuroimaging. 1000 stoke patients were evaluated for hyponatremia (serum sodium <130 meq/l). The data was analysed using Chi-square test using SPSS (Statistical package for social science) software. Results: Out of 1000 patients, 353 patients had hyponatremia. Out of this 353 patients, 238 (67%) had SIADH and 115 (33%) had CSWS. SIADH was seen in 83 patients who had ischemic stroke and 155 patients of hemorrhagic stroke. CSWS was found in 38 patients with ischemic stroke and 77 patients with hemorrhagic stroke. Statistical analysis revealed that hyponatremia significantly affects the outcome of stroke especially when it is due to CSWS rather than SIADH. Conclusion: Incidence of hyponatremia in our study population was 35%. In patients of hyponatremia 67% were having SIADH and 33% were having CSWS. Overall hyponatremia affected the outcome of stroke especially when caused by CSWS. Therefore close monitoring of serum sodium must be done in all patients who are admitted with stroke and efforts must be made to determine the cause of hyponatremia, in order to properly manage such patients thereby decreasing the mortality rate.
  5,021 169 1
Pregnancy related symptomatic vertebral hemangioma
Meena Gupta, Rajeev Nayak, Hukum Singh, Geeta Khwaja, Debashish Chowdhury
January-March 2014, 17(1):120-122
DOI:10.4103/0972-2327.128577  PMID:24753678
Vertebral hemangiomas are benign vascular tumors of the spine that remain asymptomatic in most cases and incidentally encountered on imaging. Rarely, altered hemodynamic and hormonal changes during pregnancy may expand these benign lesions resulting in severe cord compression. The management of symptomatic vertebral hemangioma during pregnancy is controversial as modalities like radiotherapy and embolization are not suitable and surgery during pregnancy has a risk of preterm labor. Few cases of pregnancy related symptomatic vertebral hemangioma with marked epidural component have been reported in the literature. We report a case of 23-year-old primigravida who developed rapidly progressive paraparesis at 28 weeks of gestation and spine magnetic resonance imaging (MRI) revealed upper thoracic vertebral hemangioma with extensive extra-osseous extension and spinal cord compression. Laminectomy and surgical decompression of the cord was performed at 32 weeks of the pregnancy. There was significant improvement in muscle power after a week of surgery. Six weeks postoperatively she delivered a full term normal baby with subsequent improvement of neurologic deficit. Repeat MRI of dorsal spine performed at 3 months postoperatively showed reduced posterior and anterior epidural components of vertebral hemangioma.
  4,993 66 1
An interesting case of metabolic dystonia: L-2 hydroxyglutaric aciduria
Padma Balaji, V Viswanathan, Amarnath Chellathurai, Debasis Panigrahi
January-March 2014, 17(1):97-99
DOI:10.4103/0972-2327.128565  PMID:24753671
L-2-hydroxyglutaric aciduria (L-2-HGA), a neurometabolic disorder caused by mutations in the L-2 hydroxyglutarate dehydrogenase (L-2-HGDH) gene, presents with psychomotor retardation, cerebellar ataxia, extrapyramidal symptoms, macrocephaly and seizures. Characteristic magnetic resonance imaging findings include subcortical cerebral white matter abnormalities with T2 hyperintensities of the dentate nucleus, globus pallidus, putamen and caudate nucleus. The diagnosis can be confirmed by elevated urinary L-2 hydroxyglutaric acid and mutational analysis of the L-2-HGDH gene. We report two siblings with dystonia diagnosed by classical neuroimaging findings with elevated urinary 2 hydroxyglutaric acid. Riboflavin therapy has shown promising results in a subset of cases, thus highlighting the importance of making the diagnosis in these patients.
  4,430 116 1
Seizure frequency and severity: How really important are they for the quality of life of patients with refractory epilepsy
Ekaterina Ivanova Viteva
January-March 2014, 17(1):35-42
DOI:10.4103/0972-2327.128544  PMID:24753657
Introduction: The data in the scientific literature about the significance of seizure severity and frequency for the quality of life (QOL) of patients with refractory epilepsy (RE) are contradictory. Objective: Our objective was to assess the impact of the seizure severity and frequency on the QOL of Bulgarian patients with RE. Materials and Methods: A total of 70 patients with RE were studied by examining the medical documentation and seizure diaries. All study participants completed quality of life epilepsy inventory (QOLIE-89). Seizure severity of only 59 patients who had a seizure in the last month was assessed by the Liverpool seizure severity scale. Results: A limited negative impact of the seizure severity and frequency on some aspects of the physical health, epilepsy, all aspects of the social health and epilepsy and the overall QOL has been demonstrated. A weak to moderate reverse correlation between the specified factors and the respective QOLIE-89 subscales has been found. Conclusion: The clinical factors seizure severity and seizure frequency have a limited negative impact mostly on the social aspects of QOL. The study results support the multidisciplinary approach to persons with epilepsy.
  4,417 112 1
Role of vascular endothelial growth factor and other growth factors in post-stroke recovery
Tanu Talwar, Madakasira Vasantha Padma Srivastava
January-March 2014, 17(1):1-6
DOI:10.4103/0972-2327.128519  PMID:24753650
Stroke is a major health problem world-wide and its burden has been rising in last few decades. Until now tissue plasminogen activator is only approved treatment for stroke. Angiogenesis plays a vital role for striatal neurogenesis after stroke. Administration of various growth factors in an early post ischemic phase, stimulate both angiogenesis and neurogenesis and lead to improved functional recovery after stroke. However vascular endothelial growth factors (VEGF) is the most potent angiogenic factor for neurovascularization and neurogenesis in ischemic injury can be modulated in different ways and thus can be used as therapy in stroke. In response to the ischemic injury VEGF is released by endothelial cells through natural mechanism and leads to angiogenesis and vascularization. This release can also be up regulated by exogenous administration of Mesenchymal stem cells, by various physical therapy regimes and electroacupuncture, which further potentiate the efficacy of VEGF as therapy in post stroke recovery. Recent published literature was searched using PubMed and Google for the article reporting on methods of up regulation of VEGF and therapeutic potential of growth factors in stroke.
  4,328 161 4
Balint syndrome
S Deepak Amalnath, Sai Kumar, S Deepanjali, Tarun Kumar Dutta
January-March 2014, 17(1):10-11
DOI:10.4103/0972-2327.128526  PMID:24753652
We report a patient who presented with complaints of blindness following stroke and was subsequently diagnosed to have Balint syndrome
  4,144 142 -
Acute flaccid paralysis due to West nile virus infection in adults: A paradigm shift entity
Boby Varkey Maramattom, Geetha Philips, Nittur Sudheesh, Govindakarnavar Arunkumar
January-March 2014, 17(1):85-88
DOI:10.4103/0972-2327.128561  PMID:24753667
Three cases of acute flaccid paralysis (AFP) with preceding fever are described. One patient had a quadriparesis with a florid meningoencephalitic picture and the other two had asymmetric flaccid paralysis with fasciculations at the onset of illness. Magnetic resonance imaging in two cases showed prominent hyperintensitities in the spinal cord and brainstem with prominent involvement of the grey horn (polio-myelitis). Cerebrospinal fluid (CSF) polymerase chain reaction was positive for West Nile virus (WNV) in the index patient. All three cases had a positive WNV immunoglobulin M antibody in serum/CSF and significantly high titer of WNV neutralizing antibody in serum, clearly distinguishing the infection from other Flaviviridae such as Japanese encephalitis. WNV has been recognized in India for many decades; however, AFP has not been adequately described. WNV is a flavivirus that is spread by Culex mosquitoes while they take blood meals from humans and lineage 1 is capable of causing a devastating neuro-invasive disease with fatal consequences or severe morbidity. We describe the first three laboratory confirmed cases of WNV induced AFP from Kerala and briefly enumerate the salient features of this emerging threat.
  4,077 139 -
A retrospective study of toddlers with autism spectrum disorder: Clinical and developmental profile
Prahbhjot Malhi, Pratibha Singhi
January-March 2014, 17(1):25-29
DOI:10.4103/0972-2327.128537  PMID:24753655
Objective: To retrospectively examine the developmental and clinical characteristics of children with autism spectrum disorders (ASD) in the first 2 years of life in order to narrow the interval between parental concern and getting a reliable diagnosis of autism. Materials and Methods: The case records of 21 children in whom a diagnosis of ASD was made in the first 2 years of life and confirmed 6 months to 1 year later were examined. The inclusion criterion was absence of neurological, metabolic, or genetic disorders and sensory or motor impairments. These case records were maintained in the Pediatric Psychology Clinic at the Department of Pediatrics of a tertiary care teaching hospital in North India. Results: The average age at presentation to the clinic was 21.23 months (SD = 2.18). The clinical characteristics that were found in two-thirds or more children included lack of speech, inability to follow verbal commands, lack of pretend play, no index finger pointing, difficulty in playing with toys in a constructive manner, lack of joint attention, and motor stereotypies. The mean IQ was 66.62 (SD = 15.11) and the mean SQ as measured by the Vineland Social Maturity Scale was 80.43 (SD = 17.45). Conclusions: Given the validity of early diagnosis over time, clinicians should be encouraged not only to make an early diagnosis but also to initiate early interventions in children with ASD.
  3,960 162 -
Alzheimer disease: An interactome of many diseases
Balaji S Rao, Krishna Kant Gupta, Pujitha Karanam, Anusha Peruri
January-March 2014, 17(1):48-54
DOI:10.4103/0972-2327.128551  PMID:24753659
Alzheimer Disease (AD) is an outcome as well as source of many diseases. Alzheimer is linked with many other diseases like Diabetes type 2, cholesterolemia, hypertension and many more. But how each of these diseases affecting other is still unknown to scientific community. Signaling Pathways of one disease is interlinked with other disease. But to what extent healthy brain is affected when any signaling in human body is disturbed is the question that matters. There is a need of Pathway analysis, Protein-Protein interaction (PPI) and the conserved interactome study in AD and linked diseases. It will be helpful in finding the potent drug or vaccine target in conscious manner. In the present research the Protein-Protein interaction of all the proteins involved in Alzheimer Disease is analyzed using ViSANT and osprey tools and pathway analysis further reveals the significant genes/proteins linking AD with other diseases.
  3,982 133 1
Babinski the great: Failure did not deter him
Man Mohan Mehndiratta, Kalyan B Bhattacharyya, Vikram Bohra, Swapan Gupta, Ankur Wadhwa
January-March 2014, 17(1):7-9
DOI:10.4103/0972-2327.128522  PMID:24753651
Joseph Babinski (1857-1932) was born on November 17, 1857. He worked in a clinical arena dominated by Charcot and a focus on hysteria. His primary aim was in trying to find the reliable clinical signs to distinguish organic from non-organic disease of the nervous system. He was considered as masterly diagnostician, relying considerably less on neuropathological reports. Babinski's first attention to the reflex of the toes occurred during a chance observation of the contrasting responses between two female patients, one a hysteric and the other a hemiplegic. He first published description of his famous "sign" in 1896. Babinski's love for research works could be gauzed from his desire to publish and by the age of 27 years, he had to this credit, 12 important articles, mainly concerned with histological and neurological themes and one of his articles on the basic description of muscle spindles was considered to be a significant one. He was awarded the doctorate degree in 1885. Babinski introduced the concept of pithiatism, meaning "curable by suggestion." He anticipated the emergence of neurosurgery in France and only 6 days prior to his death he is on record to have said that his most vital contribution to the cause of neurosciences was not the sign he described, but that he could goad Clovis Vincent and Martel to take up neurosurgery as a specialty.
  3,932 174 -
Visuo-cognitive skill deficits in Alzheimer's disease and Lewy body disease: A comparative analysis
Xuemei Li, Priyanka Rastogi, Jeffrey A Gibbons, Suprakash Chaudhury
January-March 2014, 17(1):12-18
DOI:10.4103/0972-2327.128530  PMID:24753653
Dementia is a chronic neurodegenerative disorder characterized by progressive cognitive loss. Alzheimer's disease (AD) and the Lewy body disease are the two most common causes of age-related degenerative dementia. Visuo-cognitive skills are a combination of very different cognitive functions being performed by the visual system. These skills are impaired in both AD and dementia with Lewy bodies (DLB). The aim of this review is to evaluate various studies for these visuo-cognitive skills. An exhaustive internet search of all relevant medical databases was carried out using a series of key-word applications, including The Cochrane Library, MEDLINE, PSYCHINFO, EMBASE, CINAHL, AMED, SportDiscus, Science Citation Index, Index to Theses, ZETOC, PEDro and occupational therapy (OT) seeker and OT search. We reviewed all the articles until March 2013 with key words of: Visual skills visual cognition dementia AD, but the direct neurobiological etiology is difficult to establish., Dementia of Lewy body disease. Although most studies have used different tests for studying these abilities, in general, these tests evaluated the individual's ability of (1) visual recognition, (2) visual discrimination, (3) visual attention and (4) visuo-perceptive integration. Performance on various tests has been evaluated for assessing these skills. Most studies assessing such skills show that these skills are impaired in DLB as compared with AD. Visuo-cognitive skills are impaired more in DLB as compared with AD. These impairments have evident neuropathological correlations, but the direct neurobiological etiology is difficult to establish.
  3,738 98 -
An unusual cause of ischemic stroke - Cerebral air embolism
Vinit Suri, Rohan Gupta, Girraj Sharma, Kunal Suri
January-March 2014, 17(1):89-91
DOI:10.4103/0972-2327.128562  PMID:24753668
Air embolism is a preventable, often undiagnosed but potentially treatable cause of ischemic stroke with a high morbidity and mortality. It is usually iatrogenic ocurring especially in patients in ICU setting. We describe the case and neuroimaging of a patient with ischaemic stroke due to air embolism during manipulation of central venous line. We also review the literature with respect to aetiology, incidence pathophysiology, diagnosis, and treatment options for venous and air embolism. Cerebral air embolism should be considered in patients with sudden neurological deterioration after central venous or arterial manipulations or certain neurological procedures. Prevention, as well as early diagnosis and management, may reduce morbidity and mortality.
  3,665 91 -
Cerebral tubercular thrombophlebitis presenting as venous infarct: Magnetic resonance imaging and pathologic correlation
Sandhya Mangalore, Sunali Desai, Anita Mahadevan, Jerry M. E. Kovoor, MK Vasudev, Arun Bhagwandas Tally, Susarla Krishna Shankar
January-March 2014, 17(1):130-134
DOI:10.4103/0972-2327.128587  PMID:24753682
Central nervous system involvement by tuberculosis to produce basal meningitis, hydrocephalus, arteritis and infarcts is well-known, the brunt of the pathology being borne by the arterial vasculature to produce neurological sequelae. However, tuberculous thrombophlebitis causing venous infarction is exceedingly rare. We present imaging and pathological features of two autopsy proven cases of tuberculous thrombophlebitis with venous infarcts involving superficial venous system in one and deep venous system in the other. This is the first study presenting radiopathologic correlation of this rare complication. Tuberculous thrombophlebitis should be suspected if basal exudates and multiple white matter T2 hyperintensities are seen on neuroimaging and the imaging protocol should include both magnetic resonance arteriogram and venogram.
  3,442 81 1
Multiple cerebral infarctions with severe multi-organ dysfunction following multiple wasp stings
Mushtaq Wani, Sheikh Saleem, Sawan Verma, Irfan Yousuf, Maqbool Wani, Ravouf Asimi, Riyaz Ahmed Daga, Irfan Shah, Aejaz
January-March 2014, 17(1):125-127
DOI:10.4103/0972-2327.128581  PMID:24753680
Wasp and bee sting are commonly encountered worldwide. Local reactions are more common, generally are self-limiting and settle within a few hours. Multiple stings can lead to various clinical manifestations like vomiting, diarrhea, dyspnea, generalized edema, hypotension, syncope, acute renal failure, and even death. Rarely, they can cause vasculitis, serum sickness, neuritis, and encephalitis. We are reporting a case of 40-year-old male who presented with stroke, right hemiparesis with severe multi-organ dysfunction due to multiple wasp stings.
  3,378 61 1
Clinical characteristics and long-term outcome of surgery for hypothalamic hamartoma in children with refractory epilepsy
Sita Jayalakshmi, Manas Panigrahi, Rajesh Reddy, Shanmukhi Somayajula
January-March 2014, 17(1):43-47
DOI:10.4103/0972-2327.128547  PMID:24753658
Context: Hypothalamic hamartomas (HH) are ectopic masses of neuronal and glial tissue most commonly presenting with medically refractory gelastic seizures with evolution to other seizure types. They are also associated with cognitive and behavioral problems to varying extent. Surgery has been found to improve quality of life in more than 50% of patients. Aim: To evaluate the clinical characteristics and long-term outcome of surgery in children with HH and refractory epilepsy. Materials and Methods: A retrospective analysis of presurgical, surgical, and postsurgical data of six children who underwent surgery for HH and had at least 3 years follow-up was performed. Results: Six children (male: female = 5:1) aged 3-16 years (at the time of surgery) underwent surgical resection of HH for refractory epilepsy. At last follow-up (range 3-9 years), three children were in Engel's class I, two in Class II, and one in class III outcome. Significant improvement in behavior, quality of life was noted in four children; while the change in intelligence quotient (IQ) was marginal. Conclusions: Medically refractory epilepsy associated with behavioral and cognitive dysfunction is the most common presentation of HH. Open surgical resection is safe with favorable outcome of epilepsy in 50% with significant improvement in behavior and marginal change in cognitive functions.
  3,213 79 2
A simple bedside test to assess the swallowing dysfunction in Parkinson's disease
S Vinoth Kanna, K Bhanu
January-March 2014, 17(1):62-65
DOI:10.4103/0972-2327.128556  PMID:24753662
Background: Swallowing changes are common in Parkinson's disease (PD). Early identification is essential to avoid complications of aspiration. Objectives: To evaluate the swallowing ability of the PD patients and to correlate it with the indicators of disease progression. Materials and Methods: A total of 100 PD patients (70 males and 30 females) aged between 50 years and 70 years with varying stage, duration, and severity were enrolled in a cross-sectional study carried out between January and May 2012. A simple bedside water swallowing test was performed using standard 150 ml of water. Swallowing process was assessed under three categories-swallowing speeds (ml/s), swallowing volume (ml/swallow) and swallowing duration (s/swallow). Equal number of age and sex matched controls were also evaluated. Results: All of them completed the task of swallowing. A mean swallowing speed (27.48 ml/s), swallowing volume (28.5 ml/s), and swallowing duration (1.05 s/swallow) was established by the control group. The PD patients showed decreased swallowing speed (7.15 ml/s in males and 6.61 ml/s in females), decreased swallowing volume (14.59 ml/swallow and 14 ml/swallow in females), and increased swallowing duration (2.37 s/swallow and 2.42 s/swallow) which are statistically significant. There was a significant positive correlation between the severity, duration, and staging of the disease with the swallowing performance and a poor correlation between the subjective reports of dysphagia and the objective performance on water swallow test. Conclusion: The water swallowing test is a simple bedside test to identify the swallowing changes early in PD. It is recommended to do the test in all PD Patients to detect dysphagia early and to intervene appropriately.
  3,130 112 1
Acanthamoeba meningoencephalitis
SR Chandra, Sikandar Adwani, Anitha Mahadevan
January-March 2014, 17(1):108-112
DOI:10.4103/0972-2327.128571  PMID:24753675
Report of a case of young immunocompetent male adult with autopsy proven acanthamoeba meningoencephalitis. The patient presented with a protracted febrile illness of 3 months duration with features of meningoencephalitis, this was followed by rapid deterioration while on anti tuberculous therapy and steroids and ended fatally. His magnetic resonance imaging showed features of hemorrhagic meningoencephalitis and magnetic resonance spectroscopy showed choline peak. Autopsy revealed necrotizing meningoencephalitis and intraocular colonization due to acanthamoeba.
  3,146 80 1
Relationship between activities of daily living and cognitive ability in a sample of older adults with heterogeneous educational level
Daniel C Mograbi, Camila de Assis Faria, Helenice Charchat Fichman, Emylucy Martins Paiva Paradela, Roberto Alves Lourenço
January-March 2014, 17(1):71-76
DOI:10.4103/0972-2327.128558  PMID:24753664
Introduction: A number of studies have shown the impact of cognitive abilities on instrumental activities of daily living, in particular executive functions. Nevertheless, it is not clear to what extent these results can be generalized, given that most samples studied have not included people with a low educational level. Objectives: The current study aims to investigate the association between cognitive abilities and activities of daily living in older adults - with and without dementia - from a middle-income country. Sample: The sample consisted of 48 healthy older adults and 29 people with dementia, who were evaluated in an Outpatient Care Unit in a University Reference Center in Rio de Janeiro. Results: Regression analyses indicated that the best predictors for activities of daily living were performance in immediate verbal memory in the case of controls and in a categorical fluency task in the patient group. The educational level itself was not a significant predictor of functional ability in either sample, but showed moderate correlation with the predictors. Conclusions: These results suggest that educational level may be a mediating factor in the association of cognitive variables and activities of daily living, and indicate a potential dissociation in terms of predictors according to the diagnostic status, pointing to relevant treatment directions.
  3,042 50 -
A clinical and radiological profile of neuromyelitis optica and spectrum disorders in an Indian cohort
Kavita Sohan Barhate, Malti Ganeshan, Bhim Sen Singhal
January-March 2014, 17(1):77-81
DOI:10.4103/0972-2327.128559  PMID:24753665
Background: There is insufficient data on the clinical and radiological features of neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) from India. Objective: The objective of the following study is to examine the clinico-radiological features of NMO and NMOSD in an Indian cohort. Materials and Methods: This retrospective study included 44 consecutive patients who (1) satisfied the 2006 Wingerchuk criteria for NMO (16 seropositive and 7 seronegative); or (2) had isolated or recurrent optic neuritis (ON) with seropositivity (n = 4); or (3) had isolated or recurrent myelitis with seropositivity (n = 17). Results: The female:male ratio was 7.8:1 with median age of onset 26.5 (range 8-72). Annualized relapse rate (ARR) was comparable across all groups (F [3, 40] = 0.938 and P = 0.431). Various presentations other than ON and myelitis were noted. All 40 patients with myelitis had spinal cord lesions involving ≥3 vertebral segments during the course of the disease. Cervicomedullary involvement was seen in 32.5% (13/40) patients. Brain magnetic resonance imaging was available for 40 patients; eight of these (20%) had brain lesions in locations described in multiple sclerosis (MS), 27.5% (11/40) had lesions at sites unusual for MS and 52.5% (21/40) had normal brain imaging. Conclusion: NMO and NMOSD patients in this cohort have comparable ARR regardless of clinical presentation, supporting the emerging trend of treating all patients with immunotherapeutic agents at an early stage. Varied presentations seen in NMO and NMOSD highlight the need for a high index of suspicion for NMO in demyelinating episodes not classical for MS.
  2,804 217 -
Atypical Wernicke's syndrome sans encephalopathy with acute bilateral vision loss due to post-chiasmatic optic tract edema
Soaham Dilip Desai, Diva Sidharth Shah
January-March 2014, 17(1):103-105
DOI:10.4103/0972-2327.128567  PMID:24753673
A middle aged male presented with acute bilateral vision loss, 4 weeks after undergoing gastric bypass surgery for gastric carcinoma. He had normal sensorium, fundoscopy, normal pupillary reaction to light, but had mild opthalmoparesis and nystagmus with ataxia. Magnetic resonance imaging of the brain revealed post-chiasmatic optic tract edema along with other classical features of Wernicke's syndrome. Thiamine supplementation leads to complete resolution of clinical as well as imaging findings. In appropriate clinical settings, a high index of suspicion and early treatment are essential for managing Wernicke's syndrome even in patients with atypical clinical and imaging presentation.
  2,675 66 1
Familial palmaris brevis spasm syndrome
Prasanna Venkatesan Eswaradass, Ramadoss Kalidoss, Balakrishnan Ramasamy, Gnanagurusamy Gnanashanmugham
January-March 2014, 17(1):141-142
DOI:10.4103/0972-2327.128597  PMID:24753688
  2,676 50 1
Clinical, hematological, and imaging observations in a 25-year-old woman with abetalipoproteinemia
Madhu Nagappa, Parayil S Bindu, Sikandar Adwani, Sangeeta K Seshagiri, Jitender Saini, Sanjib Sinha, Arun B Taly
January-March 2014, 17(1):113-116
DOI:10.4103/0972-2327.128574  PMID:24753676
Abetalipoproteinemia is an uncommon cause of ataxia and retinitis pigmentosa (RP). Most of the neurological and ocular manifestations occur secondary to deficiency syndromes that is consequent to fat malabsorption from the small intestine. In this report, we have described the phenotype of a young adult female who manifested with recurrent diarrheal illness in her first decade, followed by anemia, RP, and neurological involvement with progressive deafness, cerebellar and sensory ataxia, and subclinical neuropathy in her second decade of life. While RP and sensory ataxia due to vitamin E deficiency are well-recognized features of abetalipoproteinemia, deafness is rarely described. In addition, we have highlighted the abnormal posterior column signal changes in the cervical cord in this patient. Early recognition avoids unnecessary investigations and has a potential to retard the disease progression by replacing some of the deficient vitamins.
  2,554 74 -
Reversible electrophysiological abnormalities in hypokalemic paralysis: Case report of two cases
CM Sharma, Kunal Nath, Jigar Parekh
January-March 2014, 17(1):100-102
DOI:10.4103/0972-2327.128566  PMID:24753672
Compound muscle action potential (CMAP) amplitude declines during a paralytic attack in patients with hypokalemic periodic paralysis (HPP). However, serial motor nerve conduction studies in hypokalemic paralysis have not been commonly reported. We report two cases with hypokalemic paralysis, who had severely reduced CMAPs in all motor nerves at presentation during the episode of quadriparesis. However, the amplitude of CMAPs increased and reached normal levels, as the serum potassium concentration and motor power returned to normal state.
  2,420 88 1
Rare ocular manifestation in a case of West Nile virus meningoencephalitis
Konark Malhotra, Ramnath Santosh Ramanathan, Andrea Synowiec, Sandeep Rana
January-March 2014, 17(1):95-96
DOI:10.4103/0972-2327.128564  PMID:24753670
West Nile Virus (WNV) is an arthropod-borne flavivirus, which causes flu-like illness and is sporadically associated with encephalitis. Transmission to humans occurs following a bite from an infected mosquito, which acquires the virus after feeding on dead birds. WNV meningoencephalitis is a rare infection with a neuroinvasive disease occurring in less than 1% of the cases, with varied presentations including aseptic meningitis, meningoencephalitis, and acute flaccid paralysis. Chorioretinitis is the most common eye finding in this infection, while other ocular manifestations have been rarely reported in the literature. We present the first case report of WNV meningoencephalitis, with rare ocular manifestations of acute hemorrhagic conjunctivitis, bilateral subconjunctival hemorrhages, and nystagmus. The rare ocular findings of acute hemorrhagic conjunctivitis, bilateral subconjunctival hemorrhages, and nystagmus diagnosed in our case can guide clinicians toward early diagnosis of WNV meningoencephalitis, while serologic testing is still pending.
  2,417 51 -
Gender influence on selection and outcome of deep brain stimulation for Parkinson's disease
Shyambabu Chandran, Syam Krishnan, Ravi Mohan Rao, S Gangadhara Sarma, P Sankara Sarma, Asha Kishore
January-March 2014, 17(1):66-70
DOI:10.4103/0972-2327.128557  PMID:24753663
Background: Gender differences exist in Parkinson's disease (PD), both in clinical manifestations and response to medical treatment. We investigated whether gender differences occur in the clinical characteristics of patients selected for bilateral subthalamic nucleus deep brain stimulation (STN DBS) or in the outcome when resource limits influence treatment choices made by patients. Materials and Methods: Fifty-one consecutive patients were evaluated 1 month before, and 12 months after bilateral STN DBS. All patients were rated using Unified Parkinson's Disease Rating Scale, Parkinson's Disease Quality of Life (PDQL) Scale, Addenbrooke's Cognitive Examination and Beck Depression Inventory. Results: Pre-operative characteristics did not differ between the genders except for lower doses of drugs (P = 0.03), worse emotional scores in PDQL (P = 0.01) and worse depression (P = 0.03) in women. There was no gender difference in the surgical outcome, except a lesser reduction of dopaminergic drugs in women. Depression and quality of life (QOL) improved equally well in women and men. Conclusion: Bilateral STN DBS is equally efficacious in both genders as a treatment for motor complications of PD and for improving QOL. Women are likely to be undertreated because of more severe dyskinesia and may experience less emotional well-being, and could therefore potentially benefit from earlier surgical treatment.
  2,223 61 -
Glutamate-based magnetic resonance spectroscopy in neuroleptic malignant syndrome
Atri Chatterjee
January-March 2014, 17(1):123-124
DOI:10.4103/0972-2327.128579  PMID:24753679
Glutamate neurotoxicity is implicated in a number of neurological diseases, including Neuroleptic Malignant syndrome. Therefore, functional magnetic resonance imaging can help in diagnosis and monitoring such conditions. However, reports of this application are scarce in the literature. In this manuscript, glutamate based imaging of the basal ganglia showed increased levels of the neurotransmitter bilaterally. In addition, a radon transform of the functional image was performed to look for any asymmetry in cerebral activation. Although no asymmetry was detected in this case, this novel analysis can be applied in physiological and pathological scenarios to visualize contribution of different brain structures.
  2,088 47 1
Can residents diagnose this condition on X-ray?
Amita Malik, Binit Sureka, Ranjan Chandra, Rohini Gupta
January-March 2014, 17(1):135-136
DOI:10.4103/0972-2327.128589  PMID:24753683
  2,005 94 -
Essential thrombocythemia: Rare cause of chorea
Eswaradass Prasanna Venkatesan, Kalidoss Ramadoss, Ramasamy Balakrishnan, B Prakash
January-March 2014, 17(1):106-107
DOI:10.4103/0972-2327.128569  PMID:24753674
Essential thrombocythemia (ET) is a clonal myeloproliferative disorder (MPD), characterized predominantly by a markedly elevated platelet count without known cause. It is rare hematological disorder. In ET clinical picture is dominated by a predisposition to vascular occlusive events and hemorrhages. Headache, transient ischemic attack, stroke, visual disturbances and light headedness are some of the neurological manifestations of ET. Here, we describe a 55 year-old female who presented to us with generalized chorea. On evaluation, she was found to have thrombocytosis. After ruling out the secondary causes of thrombocytosis and other MPD we confirmed diagnosis of ET in her by bone marrow studies. Polycythemia vera (PV) another MPD closely related to ET may be present with generalized chorea. There are few case reports of PV presenting as chorea in the literature, but none with ET. We report the first case of ET presenting as generalized chorea.
  2,020 62 -
Vogt-Koyanagi-Harada syndrome presenting with encephalopathy
Alireza E Naeini, Dana Daneshmand, Farzin Khorvash, Ahmad Chitsaz
January-March 2014, 17(1):128-129
DOI:10.4103/0972-2327.128585  PMID:24753681
Vogt-Koyanagi-Harada (VKH) is a rare syndrome affecting tissues containing melanocytes. The possibility of its autoimmune pathogenesis is supported by high frequent HLA-DR4 presentation, commonly associated with other autoimmune diseases. Eyes are the main affected organs, resulting in blindness. Brain disease is a late-onset event, and is extremely rare. Here, we are reporting a 57-year-old woman, a known case of VKH syndrome, presenting with brain encephalopathy several decades after the initial presentation. We think this long period between initial presentation and presentation of encephalopathy due to VKH syndrome has not been described before. She was treated with corticosteroids and discharged home with a good general condition.
  1,836 65 -
Reversible conduction failure in acute motor axonal neuropathy
Sreejith Peediackal, James Jose, V Abdul Gafoor, B Smitha
January-March 2014, 17(1):142-143
DOI:10.4103/0972-2327.128601  PMID:24753690
  1,750 46 -
Mycotic aneurysms of the intracranial and peripheral circulation: A rare complication of bacterial endocarditis
Rajendra Singh Jain, Tarun Mathur, Trilochan Srivastava, Rahul Jain, Raghavendra Bakki Sannegowda
January-March 2014, 17(1):82-84
DOI:10.4103/0972-2327.128560  PMID:24753666
  1,650 66 -
Peripheral symmetrical gangrene in meningitis
Masaraf Hussain, SR Sharma, Mesasheba Rupsi
January-March 2014, 17(1):140-141
DOI:10.4103/0972-2327.128595  PMID:24753687
  1,513 55 -
Staphylococcus aureus tropical pyomyositis induced Guillain-Barré syndrome
Amita Narendra Bhargava, Subhakaran Khichar, Gaurav Mansukhlal Kasundra, Bharat S. K. Bhushan
January-March 2014, 17(1):139-139
DOI:10.4103/0972-2327.128594  PMID:24753686
  1,521 46 -
Mid life modifiable risk factors for late life Alzheimer's disease
Chetana Sen, Krishnarpan Chatterjee
January-March 2014, 17(1):138-138
DOI:10.4103/0972-2327.128592  PMID:24753685
  1,490 48 -
A rare presentation of methanol toxicity
Nikhil Gupta, Ajinkya Ashok Sonambekar, Sunil Kumar Daksh, Laxmikant Tomar
January-March 2014, 17(1):142-142
DOI:10.4103/0972-2327.128598  PMID:24753689
  1,336 54 -
Clinical neurophysiology
KA Mansukhani
January-March 2014, 17(1):144-144
  1,142 81 -
Death - My enemy
Sourabh Aggarwal
January-March 2014, 17(1):137-137
  1,026 47 -
Clinical practice of multiple sclerosis
Roop D Gursahani
January-March 2014, 17(1):145-145
  980 57 -
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