Growth and development of neuroepidemiology in India during the last four decades has been documented highlighting the historical milestones. The prevalence rates of the spectrum of neurological disorders from different regions of the country ranged from 967-4,070 with a mean of 2394 per 100000 population, providing a rough estimate of over 30 million people with neurological disorders (excluding neuroinfections and traumatic injuries). Prevalence and incidence rates of common disorders including epilepsy, stroke, Parkinson's disease and tremors determined through population-based surveys show considerable variation across different regions of the country. The need for a standardized screening questionnaire, uniform methodology for case ascertainment and diagnosis is an essential requiste for generating robust national data on neurological disorders. Higher rates of prevalence of neurological disorders in rural areas, 6-8 million people with epilepsy and high case fatality rates of stroke (27-42%) call for urgent strategies to establish outreach neurology services to cater to remote and rural areas, develop National Epilepsy Control Program and establish stroke units at different levels of health care pyramid.

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Background and Aims: Dysferlinopathies are a group of autosomal recessive muscular dystrophies caused by mutations in the dysferlin gene. This study presents clinical features and the mutational spectrum in the largest cohort of Chinese patients analyzed to date. Patients and Methods: A total of 36 unrelated Chinese patients with diagnostic suspicion of dysferlinopathy were clinically and genetically characterized. Results: Patients were divided into five phenotypes: 19 patients with limb girdle muscular dystrophy (LGMD) type 2B, 10 with Miyoshi myopathy (MM), 1 with distal anterior compartment myopathy (DACM), 3 with exercise intolerance, and 3 with asymptomatic hypercreatine phosphokinasemia (hyperCPKemia). Thirty-one patients showed an absence or drastic reduction of dysferlin expression by Westernblot. Forty-three mutations were identified in DYSF, including 31 novel. Conclusion: Our study underlines clinical heterogeneity and a high proportion of novel mutations in Chinese patients affected with dysferlinopathy.

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Background: Depression is a serious and frequent co-morbid illness among people with epilepsy (PWE) and the risk for depression in PWE is higher than the general population. However, depression among PWE is often under diagnosed and treated by physicians other than psychiatrists in resource-limited settings like Ethiopia. Objective: This study aimed at assessing the prevalence and factors associated with depression and its implication for treatment among PWE in Southwest Ethiopia. Materials and Methods: A hospital-based cross-sectional study design was facilitated among 300 adult subjects diagnosed with epilepsy at the Jimma University Specialized Hospital (JUSH) from August to October 2012. Depression was assessed using the Beck Depression Inventory-II (BDI-II) scale which was found to have Cronbach's Alpha of 0.82 for this study. Results: A total of 300 adult subjects with epilepsy were interviewed making a response rate of 100%. The prevalence of depressive disorder among patient with epilepsy was 49.3%. Of these, 39.9%, 38.5%, and 21.6% of the patient were found to have mild, moderate, and severe depression, respectively. Epilepsy-related perceived stigma, high seizure frequency, and low educational status were found to be independent predictors of depression among subjects with epilepsy. Conclusion: In this study, depression was found to be highly prevalent. Early recognition and treatment of depression through routine screening and assessment of PWE should become an essential part of diagnosis and treatment protocol.

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Background: Pharmacological treatment has mild effects for patients with Alzheimer's disease dementia (AD); therefore, the search for modifiable risk factors is an important challenge. Though risk factors for AD are widely recognized, elements that influence the time of dementia onset have not been comprehensively reported. We aimed to investigate which risk factors might be related to the age of onset of AD in a sample of patients with highly variable educational levels, taking into account the Framingham risk scoring as the sole measure of vascular risk. Subjects and Methods: We included 209 consecutive late-onset AD patients to find out which factors among educational levels, coronary heart disease risk estimated by way of Framingham risk scores, history of head trauma or depression, surgical procedures under general anesthesia, family history of neurodegenerative diseases, gender, marital status and APOE haplotypes might be related to the age of dementia onset in this sample of patients with low mean schooling. Results: Mean age of AD onset was 73.38 ± 6.5 years old, unaffected by schooling or family history of neurodegenerative diseases. Patients who were APOE-ε4 carriers, married, or with history of depression, had earlier onset of AD, particularly when they were women. Coronary heart disease risk was marginally significant for later onset of AD. Conclusions: APOE haplotypes, marital status and history of depression were the most important factors to influence the age of AD onset in this sample. While midlife cerebrovascular risk factors may increase incidence of AD, they may lead to later dementia onset when present in late life.

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Background: Neuropathy is often an associated feature woth long-standing type II diabetes mellitus. Neuropathy may occur even in subjects with impaired glucose tolerance. Objective: To study the prevalence of neuropathy using different electrophysiological techniques in subjects with impaired glucose tolerance (IGT) and no other identifiable cause of neuropathy. Materials and Methods: The study was conducted on 30 age-matched controls and 58 subjects with impaired oral glucose tolerance test (OGTT) attending diabetic awareness. Prediabetes was defined using World Health Organization (WHO) criteria. All subjects had normal glycosylated hemoglobin HbA (1c), vitamin B12 levels, and thyroid function. Neuropathy was evaluated by nerve conduction studies (NCS) performed on one upper and both lower limbs, dorsal sural nerve, medial and lateral planter nerve conductions using conventional techniques. Neuropathy was also evaluated by autononic function tests, and quantitative sensory testing (QST). The subjects were followed up for 4 years. Results: Out of 58 subjects, 19 (32.8%) had neuropathy. Nerve conduction studies showed evidence of neuropathy in 14 (24.13%) subjects, autonomic neuropathy was detected in 8 (13.8%), and QST was found to be abnormal in 16 (27.6%) subjects. Twenty subjects (34.5%) developed diabetes mellitus in the follow-up period. Conclusions: Neuropathy was detected in 32.8% subjects with IGT. Small fiber neuropathy was most common. Of all the three parameters studied, QST was found to be most sensitive technique for the detection of neuropathy. Assessment of medial plantar and dorsal sural NCS increases the sensitivity in the detection of neuropathy.

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Epilepsy-related health outcomes remain unacceptably low in much of the developing world. According to us, it is because of the failure since long to see, and address, epilepsy beyond its preset conventional image. The objective of this paper was to highlight the presence, and influence, of these conventional practices and also to demonstrate what happened when a bold unconventional approach to address epilepsy was taken in Cambodia. Data are taken from existing published literature on epilepsy as well as our field experience during several population-based surveys conducted in Cambodia. We complimented this with our knowledge gained over this long period. It is demonstrated that epilepsy is far more important in Asia that it is currently considered to be, and also vis-à-vis other geographic regions. Pregnancy and birth-related factors carry far higher odds for epilepsy than several "highly vocal" infections. A refocus in epilepsy is required to help move from its traditional negative image to an image where epilepsy is considered a "positive-looking" disorder that is full of "opportunities;" such as availability of safe effective inexpensive treatment, etc. Stigma is a two-side entity (i.e., it is present, and it is influential), and diligence should be therefore practiced before using the stigma label. Nevertheless, psychosocial aspects shouldn't be limited to stigma or Jacoby stigma scale alone. Quality of life is a subjective phenomenon, and patients should determine directly what affects them. It is highly desirable that if we want to find newer answers to old problems in epilepsy, we need to shed our conventional approach and preset conclusions. We should choose to move toward "opportunities" visibly present in epilepsy. Our Cambodian experience demonstrates more intimately that opportunities do get identified when preset conclusions are questioned, and an approach that goes beyond expected and by default is taken.

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The site of origin of primitive neuroectodermal tumors (PNETs) is quite varied and has significant influence on the prognosis. We report a case of intracranial peripheral PNET/Ewing's sarcoma arising from the superior tentorial surface in a 13-year-old girl. Gross total excision of the tumor was done. We have discussed the distinction between central nervous system PNET (CNS PNET) and Intracranial Peripheral PNET (pPNET/ES) as their treatment and prognosis varies radically. A review of literature shows that prognosis is better in intracranial pPNET/ES.

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Background: Cervical spondylotic myelopathy, radiculopathy and myeloradiculopathy can be managed by laminoforaminotomy, or bilateral decompression using posterior approach in single or multilevel compression. Posterior endoscopic techniques allow preservation of motion segment and neural decompression without fusion. Materials and Methods: A prospective study of 50 patients of cervical compressive myelopathy with primarily posterior lesion or multilevel anterior compression with acceptable preoperative lordosis was undertaken. Any instability, significant anterior compression, and cervical myelopathy secondary to tumor, trauma, severe ossification of posterior longitudinal ligament, rheumatoid arthritis, pyogenic spondylitis, and destructive spondylo-arthropathy were excluded from the study. There were 5, 23, 12, 10 patients with 2, 3, 4, 5 vertebral body level pathologies, respectively. Results: There were 2, 4, 7, 32, and 5 patients in preoperative Nurick grade 0, 1, 2, 3, and 4, respectively with an average of 2.6 grades. All the patients improved in post-operative grading with 10, 34, and 6 patients in 0, 1, and 2 grades (average 0.92), respectively. Better outcome was observed in patients with good preoperative grade and in short segment compression on cord. There was no change in cervical Cobb angle after surgery. Follow-up ranged from 6 to 24 months (averages 19 months). There was small dural tear, minor bleeding from muscles or epidural vessels and temporary C 5 root injury in 1, 3, and 2 patients, respectively. Conclusion: Endoscopic decompression of cervical spondylotic myelopathy is a safe and an effective alternative treatment option in selected patients when pathologic changes are primarily posterior or multi level anterior lesions with acceptable preoperative lordosis.

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Paralytic rabies closely simulates Guillain-Barre syndrome or ascending myelitis often causing clinical dilemma. Two such patients were managed in our hospital whose magnetic resonance imaging (MRI) revealed characteristic findings revealing T2 hyper intensity in central spinal cord and in posterior brainstem and hypothalamus. These MRI findings are helpful in the diagnosis of rabies in appropriate setting. We also review the literature on MRI changes in paralytic rabies.

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Background: Pattern of injuries among drivers, pillion riders and co-passengers of two and four-wheeler vehicles need to be separately evaluated and addressed. Materials and Methods: A prospective study was conducted on 1545 patients (1314 males and 231 females) between 01 April, 2011 to 31 December, 2011, to evaluate the profile of head injury patients due to road traffic accidents, admitted in Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh. Proper subset of cases and controls with or without helmet, seat belt and history of alcohol intake were compared. Data was analyzed to evaluate the incidence, severity, pattern of head injury and outcome of the patients. Results: Male drivers of two-wheeler vehicular accidents (71.4%) were most commonly injured. Among helmeted patients, only 4.8% sustained severe head injuries compared to 23.7% of un-helmeted patients. Only full coverage helmets were effective in preventing head injury. Among helmeted patients with a proper chinstrap, 2.6% suffered critical injuries compared to 14% of non-strapped ones. In 142 patients, helmet was at position after the crash and only 0.7% of these sustained severe head injuries. Drunk driving was noticed among 19% and 6% of two- and four-wheeler vehicular occupants, respectively. Only 7.5% of the four-wheel vehicular occupants were wearing seat belt at the time of accident. Conclusions: Injury profile of two- and four-wheeler vehicular accident victims is entirely different. A ready supply of affordable helmets of appropriate quality and strict legislation for safety constraints is the need of the hour for road safety.

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Visual loss consequent to anterior visual pathway involvement can occur in a variety of clinical settings. In a tropical country like India, apart from the usual suspects, nutritional, infective, and toxic amblyopia have to be considered in the differential diagnosis. The mode of onset (acute/chronic), unilateral versus bilateral involvement, accompanying occular pain or the lack of it, and pattern of visual loss are some of the pointers which help to differentiate optic neuropathy clinically. The presence of concurrent neurological deficits, evidence of other systemic illnesses, and the results of serological and radiological investigations help to confirm the diagnosis. This article briefly describes the important causes of optic neuropathy in the Indian context and outlines a practical approach to management.

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Background: Mycophenolate mofetil (MMF) is an affordable and tolerable drug reported to be beneficial in the treatment of multiple sclerosis (MS). Aim: To determine efficacy of MMF as first line disease modifying drug (DMD) in 40 patients with MS seen in our demyelinating disease registry. Materials and Methods: The annualized relapse rate (ARR) for 1 year prior to starting MMF therapy and 1 year post treatment was calculated. Pre- and post-treatment expanded disability status scores (EDSS), age at onset of treatment, disease duration, and type of MS were recorded. Wilcoxon rank sum test was used for comparison of ARRs and EDSS before and after treatment. Results: Forty patients included 27 females and 13 males. Mean duration of MMF therapy was 24 months (range 14-33 months). Pre-treatment mean ARR of 0.95 was significantly different from post treatment mean ARR of 0.11 (P = 0.0001). Pre-treatment mean EDSS 3.80 (inter quartile range [IQR] 3.5-4.5) was significantly different from post-treatment mean EDSS 2.66 (IQR 1.5-3.0, P = 0.0001). No adverse effects were reported that required stopping of medication. Five patients discontinued treatment 6-11 months after starting therapy, two of whom relapsed subsequently. Conclusion: Our preliminary results support the use of MMF, a cheap and well-tolerated drug, as first line disease modifying drug in MS. Long-term results in a larger patient cohort is required for validating our preliminary conclusions.

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Colloid cysts are common cysts are often located in the anterior third ventricle and septum pellucidum location is extremely rare. Cysts in septum pellucidum can be missed at surgery because of their unusual location. We describe three patients with colloid cysts in the septum pellucidum, with two in the cavum septum pellucidum. Various surgical implications of this unusual location are enumerated.

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Objectives: Retrospective pooled analysis of data from published prospective studies and randomized phase 1 and 2 trials was done to assess efficacy and safety profile of intravenous combination therapy [glycoprotein IIb/IIIa inhibitors and IV tissue plasminogen activator (tPA)] in management of acute ischemic stroke. Materials and Methods: We searched Cochrane Central Register of Controlled Trials, MEDLINE, PubMed, and EMBASE databases; two reviewers independently selected studies reporting safety endpoints and outcome measures in acute ischemic stroke patients treated with combination therapy. tPA arm of the National Institute of Neurological Disorders and Stroke (NINDS) tPA trial was included in tPA-only group. Weighted means and proportions were calculated for numeric and categorical variables respectively. Bivariate analysis using Fisher's exact test was done to compare baseline descriptors, safety endpoints, and outcome measures. Results: Combination therapy arm included 188 patients and IV tPA arm had 218 patients. Mean National Institutes of Health Stroke Scale (NIHSS) in two groups were 12.8 and 14.6, respectively. Mean time-to-treatment was 2.3 hours in combination therapy arm and 2.55 hours in tPA arm. Treatment with combination therapy was associated with significant reduction in rate of symptomatic intracranial hemorrhage (sICH) [odds ratio (OR) 0.26, 95% cumulative incidence (CI) 0.07 0.83, P value 0.01). Difference in better functional outcome at 90 days (OR 0.87, 95% CI 0.59-1.30, P value 0.54) and death at 90 days (OR 1.16, 95% CI 0.69-1.93, P value 0.60) were not significantly different in two groups. Conclusion: Combination of low dose IV TPA with glycoprotein IIb/IIIa inhibitors is associated with reduction in sICH rates in patients with acute ischemic stroke as compared to standard dose of IV tPA.

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Background: Flow diversion is a novel method of therapy wherein an endoluminal sleeve, the flow diverter stent is placed across the neck of complex aneurysms to curatively reconstruct abnormal vasculature. We present the first Indian single center experience with the pipeline embolization device (PED) and 6 months follow-up results of 5 patients. Subjects and Methods: Five complex or recurrent intracranial aneurysms in five patients were treated with PED. The patients were followed-up with magnetic resonance angiography (MRA) after 4 weeks and conventional angiography after 6 months. Feasibility, complications, clinical outcome, early 1-month MRA and 6 months conventional angiographic follow-up results were analyzed. Results: Of the five aneurysms treated, four were in the anterior circulation and one in the posterior circulation. All five patients were treated with a single PED in each, and additionally coils were used in one patient. At 1-month MRA follow-up, complete occlusion was seen in 2 (40%) of the five cases. Post 6 months conventional angiography showed complete occlusion of the aneurysm sac in all five cases (100%). Side branch ostia were covered in three patients, all of which were patent (100%). There was no incidence of major neurological morbidity or mortality. One patient (20%) who had basilar top aneurysm experienced minor neurological disability after 5 days which partially improved. Conclusions: Pipeline embolization device for complex and recurrent aneurysms is technically feasible, safe, offers low complication rate, and definitive vascular reconstruction. PED can be used without fear of occlusion of covered eloquent side branches and perforators.

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