Aim: To assess the neurological complications of chickenpox with prognosis. Background: The neurological complications occur in 0.03% of persons who get chickenpox. There is no universal vaccination against chicken pox in India. Most patients prefer alternate modalities of treatment. Hence these complications of chickenpox are likely to continue to occur. Study Design: A prospective study was conducted for 2 years (from March 2002) on the admitted cases with neurological complications after chickenpox (with rash or scar). Patients were investigated with CT/MRI, CSF study, EEG and nerve conduction studies and hematological workup. They were followed-up for 1 year and outcome assessed using modified Rankin scale. Results: The latency for the neurological complications was 4-32 days (mean: 16.32 days). There were 18 cases: 10 adults (64%) and 8 children (36%). Cerebellar ataxia (normal CT/MRI) was observed in 7 cases (32%) (mean age: 6.85 years). One patient (6 years) had acute right hemiparesis in the fifth week due to left capsular infarct. All these cases spontaneously recovered by 4 weeks. The age range of the adult patients was 13-47 years (mean: 27 years). The manifestations included cerebellar and pyramidal signs (n-4) with features of demyelination in MRI who recovered spontaneously or with methylprednisolone by 8 weeks. Patient with encephalitis recovered in 2 weeks with acyclovir. Guillain Barre syndrome of the demyelinating type (n-2) was treated with Intravenous immunoglobulin (IVIG) and they had a slow recovery by a modified Rankin scale (mRs) score of 3 and 2 at 6 months and 1 year, respectively. One case died after hemorrhage into the occipital infarct. There were two cases of asymmetrical neuropathy, one each of the seventh cranial and brachial neuritis. Conclusion: Spontaneous recovery occurs in post-chickenpox cerebellar ataxia. Rarely, serious complications can occur in adults. The demyelinating disorders, either of the central or peripheral nervous system, can be effectively managed using methylprednisolone or I/V IG.

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The effects of an intervention is best measured in a randomized controlled trial (RCT) and can be expressed in various ways using the measures such as risk difference, number needed to treat (NNT), relative risk or odds ratio. Risk difference (RD) is the difference in risk of the outcome event between control and experimental group. Control group is not exposed to the intervention, whereas experimental group is the one that is exposed to intervention. The risk of outcome event in the control group is also called baseline risk. The NNT is the inverse of the risk difference and indicates the number of patients required to be treated to avoid one additional outcome event. Risk difference and NNT are absolute measures of effect. Relative risk (RR) is a relative measure and is the ratio of the risk in the exposed group to that in the unexposed group. Relative risk reduction (RRR) is one minus RR and indicates the fraction (or percent) of baseline risk that reduces with exposure to the intervention. Odds ratio (OR) is ratio of odds of having the event in the exposed group to that in the unexposed group. These measures are suitable for different purposes and appeal to different constituencies. Odds ratio is the only measure suitable for use in logistic regression and case control studies.

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Limb girdle muscular dystrophies (LGMD) are characterized by involvement of the pelvic and shoulder girdles, classically with an onset in the second or third decade and a slow progression as opposed to Duchenne muscular dystrophy. In fact, there are many clinical variants that are related to this broad definition. For the past 13 years and since the discovery of calpain-3 as the underlying defect in LGMD 2A in 1995, a number of different genes have been found to cause LGMD; some of whose encoding proteins are located either in the sarcolemma, nucleus, cytosol or in the extra-cellular matrix. Very little is known regarding a possible common pathogenesis between all these entities. The current nomenclature of LGMDs, although a bit confusing, is still necessary to continue the establishment of homogeneous cohorts of patients and to look for unknown genes. The diagnosis of LGMD is nowadays based on a complementary clinical, immunocytochemical and genetic approach that is best achieved in specialized myology centers. In this context, India can make a significant contribution to improve the routine diagnosis in LGMD patients and to find new LGMD genes in genetic isolates. Therapeutic prospects in LGMD, although quite exciting, remain at a preliminary stage, especially those with gene-therapy orientation.

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Neuromyelitis optica (NMO) or Devic's disease was until recently regarded as an unusual or severe variant of multiple sclerosis. However the last decade has seen major advances in our understanding of the etiopathogenesis of NMO. Though unsettled, there seems to be increasing evidence that NMO is a distinct immune mediated, largely relapsing, inflammatory, demyelinating disease of the central nervous system that most commonly targets the optic nerves and spinal cord. Longitudinally extensive spinal cord lesions on MRI, a serum antibody (NMO-IgG) which reacts specifically with water channel aquaporin 4 (AQP-4), response to immunosuppressive therapies (rather than immunomodulation like in MS) seem to be distinguish it from MS. We review the evolving epidemiological, clinical, imaging and immunological features of NMO and discuss the therapeutic options available. We also review the longstanding debate on whether NMO and Asian optico-spinal multiple sclerosis are two distinct entities or one. We have reviewed the all the published literature on NMO from India and there seems to be a higher proportion of NMO in India than reported in the West. An early accurate diagnosis and treatment with widely available drugs could alter the prognosis of NMO. A fuller understanding NMO may be the doorway to insights into MS and other demyelinating disorders. The information presented in this review has been obtained from peer reviewed publications, conference abstracts and personal experience. The data on Indian patients has been obtained by a literature search using the national library of medicine search engine with the search terms: "neuromyelitis optica or multiple sclerosis or transverse myelitis or optic neuritis or demyelination and India".

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We present a report on an 18-year-old boy with Niemann-Pick disease Type C (NP-C) who presented with progressive decline in scholastic performance since 9 years of age. At 12 years, he developed abnormal behavior and after 2 years had insidious onset, progressive gait ataxia and dysarthria followed by dystonia of the right upper extremity, excessive drooling, dysphagia and nasal regurgitation. He had coarse facies, depressed nasal bridge, high arched palate, crowded teeth, splenomegaly and peculiar facial grin. In addition, impaired vertical saccadic and pursuit eye movements, brisk muscle stretch reflexes and limb and gait ataxia were observed. He had a low IQ of 47 on Binet-Kamat test. The ultrasound examination of the abdomen confirmed the presence of moderate splenomegaly. Magnetic resonance imaging brain showed symmetrical leucoencephalopathy and mild cerebellar atrophy. Bone marrow aspiration showed numerous foamy macrophages and sea-blue histiocytes suggesting the diagnosis of NP-C.

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Wilson's disease is an inborn error of copper metabolism, which is potentially treatable. This prospective study reports 21 cases of Wilson's disease from north-west India having various clinical, biochemical and radiological features. The study highlights that the juvenile forms showed predominantly dystonic features with high frequency of seizures, while elderly group of patients showed predominantly cerebellar features. The absence of family history and relative lack of previous hepatic involvement may lead to the delay in diagnosis.

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Kleine Levin syndrome is a rare syndrome that involves hypersomnolence, megaphagia and psychiatric disturbances of unknown etiology and pathogenesis. A 16-year-old male with tuberculous meningitis developed hypersomnia and megaphagia with cranial magnetic resonance imaging (MRI) showing increased T2 hyperintensity in the cerebral peduncles and left occipital cortex. Antitubercular therapy led to the disappearance of clinical symptoms and MRI changes.

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Background: Rebleeding is an important cause for mortality following aneurysmal subarachnoid hemorrhage. Early intervention is believed to reduce the risk of rebleeding. However, in developing countries such as India, early intervention is often difficult due to various reasons. The knowledge about the risks and predictors for rebleeding is essential to prioritize the management policy. Aims: To describe the frequency and impact of rebleeding in the modern era of aneurysm care, to study the clinical profile of patients with rebleed and to review the controversial aspects on aneurysmal rebleed. Materials and Methods: This observational study is based on the retrospective analysis of the case records of all patients admitted with aneurysmal subarachnoid hemorrhage (SAH) since January 1999 in our institute. Twenty patients rebled after admission before surgical clipping, and these patients formed our study group. The findings were compared with all the major series on rebleeding published in literature. Results: Since January 1999, 952 patients underwent treatment for aneurysmal subarachnoid hemorrhage in our institute. Twenty patients rebled while awaiting surgery following the admission with an incidence of 2.14%. The study group included nine males and eleven females. The age of the patients ranged from 31 to 69 years, but the majority were in the sixth decade. Mean bleed to admission days was 7.9 days (range: 1-27 days), the mean admission to rebleed was 1.42 days (Range: 0-4) and the mean first bleed to rebleed was 9.26 days. The history of loss of consciousness at the time of bleeding was observed in 16 patients. Four patients bled before an angiogram could be performed. Two patients had giant aneurysms, while the rest had small aneurysms; two patients had multiple aneurysms. Angiographic spasm was observed in eight patients. In three patients, rebleeding was precipitated by angiogram. Twelve patients rebled while taking rest or in sleep, while one person rebled while defecating. Nine patients were taken up for emergency exploration immediately after rebleeding. From these, six patients died and three survived, but were severely disabled. The remaining eleven patients who were conservatively treated expired after varying periods of intensive care. Conclusions: Our incidence of rebleeding (2.14%) is considerably less than most of the other reported series, probably related to the delayed referral pattern. Probable predisposing factors for rebleed include old age, female sex and an anterior communicating artery location. Known risk factors such as hypertension, poor neurological grade on admission, stressful activity and aneurysm morphology do not appear to be significant in our study. Outcome is extremely poor following the rebleeds, either in immediate surgery or conservative treatment.

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It is well accepted in clinical medicine that nerve conduction studies are helpful in the diagnosis of diabetic peripheral neuropathy. However, some clinicians utilize serial nerve conduction studies to make inferences regarding the progression of peripheral neuropathy. The validity of this clinical construct has not been established. In order to determine the variability in serial nerve conduction studies, sensory and motor responses were obtained from the caudal nerve of the tail of a diabetic monkey. In particular, the sensory and motor nerve conduction studies were obtained on seven separate occasions over a period of 4.5 months in a diabetic Macaca mulatta monkey. The coefficient of variation in the motor onset latency, motor amplitude, sensory onset latency and sensory amplitude was 10.7%, 56.4%, 4.2% and 23.4%, respectively. These results suggest that physicians should be cautious in making clinical inferences based on the changes in serial nerve conduction studies.

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Intracranial aspergilloma is a dreadful CNS infection with nonspecific clinical manifestation and radiological features. Therefore, delay in the diagnosis is common, resulting in a fatal outcome in almost all the cases. We present how the diagnosis of this condition is overlooked if we do not follow the conventional medical rules of taking history and thorough preoperative evaluation. These clinicoradiological points can be of help in early diagnosis and better outcomes.

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A 10-year-old child presented with neuroregression. Human immunodeficiency virus infection was confirmed. computed tomographic scan and psychological evaluation confirmed dementia. There was remarkable improvement on anti-retroviral therapy.

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