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Majority of Indians have no access to centres of neurological excellence in the country. A detailed analysis of 3666 members of the Neurological Society of India and the Indian Academy of Neurology revealed that not a single member lived in a geographical area covering 934.8 million people. 30.09% live in the four major metropolitan cities, 29.54% in the state capitals, 30.58% in Tier 2 cities, 7.12% in tier 3 cities and 2.67% in rural areas covering a population of 84.59 million. Building additional neurological centres cannot be the only answer, given the acute shortage of funds and trained personnel. In 1999, the author among others, foresaw that it could be possible, to extend the reach of urban specialists to suburban and rural India, by virtual means. The neurological community has been slow to use Information and Communication Technology (ICT) as an integral part of their health care delivery system. This article analyses the distribution of neurologists and neurosurgeons in India and suggests that providing additional virtual neurological care can be the only answer to offset the lop sided distribution of clinical care givers in neurosciences. In this article, the authors' considerable experience in introducing and developing telehealth in India over the last 15 years is being shared with specific emphasis on its relevance to neurosciences. A review of the global literature on telemedicine and neurosciences will substantiate the plea that telemedicine must be deployed by neurologists and neurosurgeons in India to extend their reach to patients particularly those residing in rural areas.

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Sher-i-Kashmir Institute of Medical Sciences (SKIMS) is the only tertiary care institute in the state of Jammu and Kashmir. The department of neurosurgery was established nearly three decades ago and continues to be the leader in providing high quality neurosurgical services in the region. The article provides an insight into the genesis of the department and its sustained growth over these years. It also describes the plans for its future development.

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Citrin deficiency is an autosomal recessive genetic disorder caused by a defect in the mitochondrial aspartate/glutamate antiporter, citrin. The disorder manifests either as neonatal intra-hepatic cholestasis or occurs in adulthood with recurrent hyperammonemia and neuropsychiatric disturbances. It has a high prevalence in the East Asian population, but is actually pan-ethnic. We report the case of a 26-year-old male patient presenting with episodes of abnormal neuro-psychiatric behavior associated with hyperammonemia, who was diagnosed to be having citrin deficiency. Sequencing of the SLC25A13 gene revealed two novel mutations, a single base pair deletion, c. 650delT (p.Phe217Serfs*33) in exon 7, and a missense mutation, c. 869T>C (p.Ile290Thr) in exon 9. Confirmation of the diagnosis allowed establishment of the appropriate management. The latter is an essential pre-requisite for obtaining a good prognosis as well as for family counseling.

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Background: Traumatic cerebrospinal fluid (CSF) rhinorrhea is a serious and potentially fatal condition as it may lead to meningitis. As acetazolamide decreases CSF production and hence CSF pressure, it has been proposed that the medication may help in curing CSF rhinorrhea. There is no definitive evidence, however, that acetazolamide is actually beneficial in treating traumatic CSF rhinorrhea. The aim of this study was to determine if the administration of acetazolamide in patients of head trauma with CSF rhinorrhea was beneficial in decreasing the duration of CSF rhinorrhea. The acid-base and electrolyte changes caused by the drug were also studied. Materials and Methods : We conducted a single center randomized prospective study. Forty-four patients of head trauma with CSF rhinorrhea were divided into two groups, the experimental group (21 patients) was given acetazolamide; and, the control group (23 patients) did not receive the medication. The median duration of CSF leak in days, and the electrolyte changes observed on administration of the medication were recorded in both the groups. Results : Both the experimental and control groups were well matched in terms of age, sex, mechanism of injury, Glasgow Coma Scale (GCS) and the type of skull fracture. The median duration of CSF leak in the control group was of 4 days and in the study group, of 5 days. Acetazolamide caused significant metabolic acidosis and hypokalemia (as shown by decreased serum pH, serum bicarbonate and serum potassium levels) in the experimental group when compared to the control group. Conclusions : Acetazolamide did not influence the resolution of traumatic CSF rhinorrhea and instead lead to significant metabolic and electrolyte disturbances.

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Object: To evaluate the outcome of patients undergoing a decompressive craniectomy (DC) in traumatic brain injury (TBI) and the factors predicting outcome. Materials and Methods: A total of 1,236 patients with TBI operated with a DC from January 2008 to December 2013 at a tertiary care hospital were included in the study. The data from the hospital computerized database was retrospectively analyzed and 324 (45%) patients were followed-up for a mean duration of 25.3 months (range 3-42 months) among the cohort of 720 alive patients. The institute's ethical committee clearance was obtained before the start of the study. Results: There were 81% males with a median age [interquartile range (IQR)] of 32 (23-45) years. The mortality rate and median (IQR) Glasgow outcome score (GOS) at discharge in patients presenting with minor, moderate, and severe head injury were 18%, 5 (4-5); 28%, 4 (1-5); and 47.4%, 2 (1-4), respectively. An overall favorable outcome (GOS 4 and 5) at discharge was observed in 46.5% patients and in 39% patients who presented with severe TBI. Only 7.5% patients were in a persistent vegetative state (PVS), while 78% had an overall favorable outcome at the last follow-up of surviving patients (P < 0.001). On multivariate analysis, the factors predictive of a favorable GOS at discharge were: a younger age (odds ratio (OR) 1.03, confidence interval (CI) = 1.02-1.04; P < 0.001), no pupillary abnormalities at admission (OR 2.28, CI = 1.72-3.02; P < 0.001), absence of preoperative hypotension (OR 1.91, CI = 1.08-3.38; P = 0.02), an isolated TBI (OR 1.42, CI = 1.08-1.86; P = 0.01), absence of a preoperative infarct (OR 3.68, CI = 1.74-7.81; P = 0.001), presence of a minor head injury (OR 6.33, CI = 4.07-9.86; P < 0.001), performing a duraplasty (OR 1.86, CI = 1.20-2.87; P = 0.005) rather than a slit durotomy (OR 3.95, CI = 1.67-9.35; P = 0.002), and, avoidance of a contralateral DC (OR 3.58, CI = 1.90-6.73; P < 0.001). Conclusions: The severity of head injury, performing a duraplasty rather than a slit durotomy, avoidance of a contralateral DC, and the presence of preoperative hypotension, infarct, and/or pupillary asymmetry have the highest odds of predicting the short term GOS at the time of discharge, after a DC in patients with TBI. Although DC carries a high risk of mortality, the probability of the survivors having a favorable outcome is significantly more as compared to those who remain in a PVS.

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Background: Central nervous system (CNS) involvement in scrub typhus is seen in up to a quarter of patients. However, the literature on cerebrospinal fluid (CSF) analysis and outcome in meningitis/meningo-encephalitis due to scrub typhus is scant. Materials and Methods: This retrospective study included patients who were admitted to a medical college hospital with scrub typhus meningitis/meningo-encephalitis between 2005 and 2011. The clinical and laboratory profile, details of CSF analysis and outcome were documented. Results: The study included 189 patients with meningitis/meningo-encephalitis due to scrub typhus. The mean age of the patients was 41 ± 4 years. The mean duration of fever before presentation was 9.4 ± 3 days. The common presenting complaints were headache (64.2%), nausea/vomiting (60%), altered sensorium (53.7%) and seizures (22.1%). The presence of an eschar was documented in 27.5% of the patients. The mean CSF white blood count was 80 cells/cu mm (range: 5-740). There was a clear lymphocyte predominance (mean 87.6%). The mean CSF protein level was 105 mg% (range: 13-640). The mean CSF sugar level was 63.9 mg% (range 25-350), and was less than 40 mg% in 11.1% of the cases. The case fatality rate was 5.8% (11/189). Univariate analysis showed the presence of an eschar (15.4% vs 2.2%; Odds Ratio [OR]: 8.1) and altered sensorium (9.8% vs 1.1%; OR: 9.2) to be significant predictors of mortality. Conclusions: In endemic regions, scrub typhus should be considered in the differential diagnosis of aseptic meningitis. Modest elevation of cells in the CSF with lymphocytic pleocytosis and multi-organ involvement may indicate scrub typhus meningitis/meningo-encephalitis.

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Objectives: In patients with neurocysticercosis, perilesional inflammatory activity is thought to be responsible for seizures. This study was designed to evaluate the serum and cerebrospinal fluid (CSF) concentrations of cytokines as well as matrix metalloproteinase (MMP)-2 and MMP-9 in patients with a solitary cysticercus granuloma. Materials and Methods: The study included 47 patients suffering from seizures in whom a solitary cysticercus granuloma was detected on a computed tomography (CT) scan. The study also included 47 control subjects. Their serum and cerebrospinal fluid (CSF) was analysed for cytokines and MMP levels. A follow-up CT was performed after 6 months. Results: The median levels of cytokines, interleukin (IL)-1β, IL-5, IL-6, IL-10 and tissue necrosis factor (TNF)-α, MMP-2 and MMP-9 were significantly elevated, both in the serum and CSF of patients having an intracerebral solitary cysticercus granuloma, in comparison to that of controls. The follow-up CT revealed that in 27 patients, the lesions were calcified and in 5 patients, there was complete resolution of the lesions. In 15 patients, the lesions remained unchanged. Higher baseline CSF MMP-2 and TNF-α level were seen in patients with persisting lesions. Higher serum baseline MMP-2, IL-6 and a low CSF IL-10 level were seen in patients with complete resolution of the granuloma. A high baseline IL-1β level was associated with a calcified lesion. Fourteen patients had recurrence of seizures. A high baseline serum TNF-α level was independently associated with seizure recurrence (P = 0.021, OR = 1.041, CI = 1.006 to 1.078). Conclusion: In patients with a solitary cysticercus granuloma, cytokines and matrix metalloproteinases in the CSF and serum are elevated. Different patterns of immunological changes were observed in patients following resolution or calcification of the lesion.

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Aim: To document the frequency and prevalence of non-motor symptoms (NMS) in an Indian cohort of Parkinson's disease (PD) patients. Objectives: To validate the non-motor score scale (NMSS) in an Indian cohort of PD patients for recording NMS of the disease, to study the prevalence and frequency of NMS in Indian PD patients using the NMSS, and to compare the progression of NMS with motor worsening. Conclusion and Results: This was a cross-sectional, single-center, open-label, one point in time evaluation study conducted from 2009 to 2011. It validated the NMSS scale in an Indian population. The study has profiled the prevalence and pattern of NMS in an Indian cohort of PD patients. Comparison of NMS scale scores with the Unified PD Rating Scale motor scores demonstrated a correlation between non-motor and motor symptoms in the disease progression, particularly of manifestations related to the cognitive decline, memory disturbances, urinary incontinence and smell.

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Clay-shoveler's fractures are isolated, avulsion-type spinous process fractures of the lower cervical and upper thoracic vertebrae. Multi-level fractures of the spinous processes are extremely rare. We report the case of a 60-year-old female patient with a six-level isolated spinous process fracture of the thoracic spine. Our case is the fourth reported case in literature, of an isolated spinous process fracture involving five or more levels in the thoracic vertebrae.

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Maximal resection of gliomas is the current standard of care. Various technical adjuncts facilitate this. Aminolevulinic acid (ALA)-induced fluorescence guided resection (FGR) is one such strategy. We review the current literature related to ALA FGR. It is based on the selective uptake of ALA into glioma cells and its preferential conversion to protoporphyrin IX. This selectivity provides a high positive predictive value for ALA induced fluorescence. Since the introduction of this technique, clinical experience supports its efficacy in improving resections in malignant gliomas when compared to other contemporary intraoperative imaging strategies such as the magnetic resonance imaging (MRI) or the adjuncts that exhibit passive permeability like fluorescein. Future research into the understanding of the basis of ALA metabolism in glioma cells and advances in visualization technology will potentially improve the scope of application of this technique.

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Context: Phenytoin (PHT) is one of the frontrunner drugs used as monotherapy in the management of epilepsy. It is also one of the most common drugs causing adverse drug reactions (ADRs). The aim of this study was to study the relationship between serum PHT levels and the age, gender, dosage and genetic polymorphisms in a North Indian population. This knowledge will help in devising drug dosage schedules in various sub-groups of patients as well as in reducing its ADRs. Materials and Methods: A retrospective analysis of data of 6224 patients from 1998 to 2009 receiving PHT alone for greater than (>) 4 weeks was performed. Patients suspected of being non-compliant, being overdosed or having a hepatic or renal disorder were excluded from the study. Two thousand eight hundred and eighty-eight patients fulfilling the inclusion criteria were divided into three groups: children (1-18 years), adults (19-60 years) and elderly (>60 years). Results: There was a male preponderance (80%) in all the groups. A significant difference was found in the mean dose between children and adults as well as between children and elderly (P = 0.00). Also, there was a significant difference in the mean concentration and dose ratio between children and adults (P = 0.00). However, a negative correlation was observed between the daily dose and dose ratio (r = -0.36, P = 0.00) that was highest (r = -0.58, P = 0.00) in the elderly. There was a significant gender difference in the mean dose in both children (P = 0.03) and adults (P = 0.00), whereas the mean concentration differed in adults only. Every fifth patient was an intermediate metabolizer (IM) (CYP2C9*1/*3) and showed higher steady state drug levels (>17 mg/L) compared with extensive metabolizers (EMs) (<12 mg/L). The genetic difference between IM and EM was more prevalent in the dose ratio at maintenance dose, with a mean ± SD of 4.041 ± 1.288 mg/L/mg/kg in nine patients carrying the CYP2C9*1/*3 genotype compared with 2.145 ± 0.817 mg/L/mg/kg in 26 patients carrying the CYP2C9*1/*1 genotype (P = 0.00). Conclusion: North Indian female children and male adults frequently attain a higher serum concentration with the same dose when compared to the other groups. Absence of poor metabolizers may be responsible for a lower number of cases exhibiting toxicity in our population; however, this needs elucidation in a larger number of patients.

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Background: The conventional laminectomy may precipitate numerous long-term complications such as the development of kypho-scoliosis, spinal instability, epidural fibrosis and loss of bony shield over the spinal cord, thus increasing the risk of a subsequent myelopathy. The present study evaluates the efficacy of a unilateral hemilaminectomy as the surgical approach of choice for excision of juxtamedullary spinal neoplasms. Materials and Methods: From January 2001 to December 2010, 83 patients (male: female ratio: 58: 25, mean age 37.4 years, median age 45 years) harboring a juxta-medullary spinal neoplasm were managed at our center. The radiological investigations included a contrast-enhanced magnetic resonance imaging (MRI) scan, (in the axial, sagittal and coronal planes). A high speed drill and an operating microscope assisted in the microsurgical excision of these neoplasms. During follow-up, MRI scans were obtained to check for any residual tumor. Results: Complete tumor excision was achieved for all patients. At a mean follow-up of 17.6 months, the patients had improved to a better clinical status. The immediate postoperative complications in the form of paraparesis and cerebrospinal fluid leak were seen in 1 patient each, respectively; while none of the patients either developed post-operative spinal instability or required conversion of the hemilaminectomy to a conventional full laminectomy. Conclusion: A unilateral hemilaminectomy is recommended as the microsurgical approach of choice for juxtamedullary spinal neoplasms.

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Aims: To evaluate the outcome of giant intra-dural aneurysms managed with endovascular techniques. Materials and Methods: We retrospectively reviewed a series of 39 consecutive giant intra-dural aneurysms. The technical feasibility of endovascular treatment, its complications, the angiographic results and the clinical outcome were assessed. Logistic regression analysis was performed to evaluate for predictors of a poor outcome. Results: Nine patients were left untreated. During a 30 month follow-up, four of them (44.4%) died and two (22.2%) deteriorated. Thirty aneurysms (12 located in the anterior circulation and 18 located in the posterior circulation) were treated using endovascular methods. Of these, 11 were treated by parent vessel occlusion, 11 by stent-assisted coiling, one using only coils, six using solely a stent, and, one using both coils and onyx. During a 28 month follow-up, seven (23.3%) patients died and two (6.7%) patients experienced permanent neurological deficits. The mortality and morbidity in the endovascular group seemed lower than that in the untreated group (P = 0.045, 30% vs. 66.7%). There was no difference in the results of endovascular treatment between giant intra-dural aneurysms located in the posterior and the anterior circulation. Conclusions: Giant intra-dural aneurysms, whether treated or not, may have a poor clinical outcome. The outcome following endovascular treatment of these lesions is better than its natural history when left untreated. However, endovascular treatment may often be associated with high complication rates and a low chance of cure.

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Aim: In this study, we genotyped eight microsatellite markers on chromosome 5 and performed linkage analyses. We aimed to establish the pathogenic gene loci in this familial cortical myoclonic tremor with epilepsy (FCMTE) pedigree. Materials and Methods: Reliable clinical information was obtained on the Chinese family members. Our study performed linkage analysis across these loci to identify and further characterize the pathogenic gene locus underlying FCMTE in Chinese patients. Results: Positive signals (>1) were only obtained for 5p15.31-p15 (Logarithm of Odds (LOD) values 2.16 and 1.34 for D5S1957 and D5S2095, respectively; θ =0.0), supporting involvement of this region in the FCTME pedigree analyzed. Conclusion: Genetic analysis of a Chinese family provides further evidence for linkage of FCMTE to 5p15.31-p15.

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