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Background: Spinocerebellar ataxia 1 (SCA1) and SCA2 are dominantly inherited ataxias caused due to CAG expansion mutation in ATXN1 (CAG≥39) and ATXN2 (CAG≥32) genes located at 6p22.3 and 12q24.12 loci, respectively, with key manifestations of progressive limb and gait ataxia and with or without brain stem and pyramidal tract involvement. Both SCA1 and SCA2 are quite prevalent subtypes among the SCAs. There are very few reports that describe a combinatorial SCA subtype mutation in a single patient. Cases: Here, we report a novel co‐occurrence of SCA1 and SCA2 mutations in two unrelated patients. Case‐1 was observed to carry ATXN1‐CAG (30/40) and ATXN2‐CAG (23/45), while case‐2 harbored ATXN1‐CAG (29/42) and ATXN2‐CAG (23/41). Overall, the clinical outcome was complex with probable early onset than expected in Case‐1 and in Case‐2, we observed a significant delayed onset of the disease than expected. Conclusion: These cases highlight the probabilistic interactive outcome of two unrelated genetic events towards a converging phenotype. [ABSTRACT FROM AUTHOR]

Introduction: Two pharmacological possibilities exist for an acute ischemic stroke (AIS): recanalization of the occluded artery and neuroprotection from ischaemic injury, the latter's efficacy being debatable. We sought to determine whether administration of Citicoline immediately after recanalization therapy for AIS would improve clinical and radiological outcome at three months compared to standard treatment alone. Patients and methods: CAISR was a single centre, randomized, placebo-controlled, parallel-group trial with blinded endpoint assessment. It was approved by the All India Institute of Medical Sciences Institutional ethics committee and registered at the Clinical Trial Registry of India (CTRI/2018/011900). We recruited participants with AIS undergoing recanalization therapy and randomly assigned them to receive either Citicoline or placebo in 1:1 ratio. Citicoline arm patients received Citicoline 1gm BD intravenously for three days, followed by oral citicoline 1gm BD for 39 days. Placebo arm patients received 100ml intravenous normal saline for three days, followed by multivitamin tablet BD for 39 days. All patients received standard of care. Outcome: Blinded assessors did the follow-up assessment at six weeks (MRI Brain-stroke volume) and three months (NIHSS 0–2, mRS 0–2 and Barthel index> = 95). Results: The infarct volume decreased from week 1 to week 6 by 2.6 cm3 on placebo versus 4.2 cm3 on Citicoline (p-0.483). The OR for achieving NIHSS 0–2, mRS 0–2 and Barthel index> = 95 with Citicoline was found to be 0.96(95%CI 0.39–2.40), 0.92(95%CI 0.40–2.05) and 0.87(95%CI 0.22–2.98) respectively. Conclusion: CAISR was the first to evaluate the role of Citicoline, when used immediately after recanalization therapy, when the penumbral tissue is the most susceptible either to be protected from injury or become ischemic. We did not find any significant difference between the Citicoline or placebo arms with respect to either our primary or secondary outcomes. [ABSTRACT FROM AUTHOR]

Background: Solitary cysticercus granuloma (SCG) appearing as a single ring-enhancing lesion (SREL) is the most frequently encountered imaging finding in patients of neurocysticercosis (NCC) in India and during follow-up, most of SCGs resolve with or without calcifications. Recurrent SCG have been rarely reported.Objectives: The aim of our study is to report the incidence of recurrent SCG in a cohort of patients with SCG and postulate the hypothesis.Materials and Methods: This retrospective study included 278 patients with SCG meeting the criteria of NCC. Their medical records and imaging studies were analyzed.Results: Out of 278 patients, 119 patients with SCG meeting the criteria of NCC with follow-up imaging were included. 15 (12.61%) had recurrent NCC and 104 (87.4%) patients did not have any recurrence during a median follow-up of 14.23 months (range; 0.24 - 113.3) and 25.26 months (range; 3.09- 98.11) respectively. Out of 15 recurrent NCC cases, ten patients had documented imaging resolution or partial regression of previous lesion followed by occurrence of new SCG in the same location, three patients had change in morphology of lesion from solitary discrete REL to solitary conglomerate REL secondary to development of new cysticercus granuloma adjacent to old lesion patients had recurrent lesions adjacent to previous lesion and two patients had new SCG in different locations following regression of old SCGs.Conclusion: Recurrent SCGs are not uncommon in NCC and recurrence is more likely to occur at the site of initial infection and therefore may be mistaken for persistent infection or other granulomatous lesions. [ABSTRACT FROM AUTHOR]

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an astrocytopathy with a predilection for the optic nerve, spinal cord, and brainstem. In this ambispective study, we evaluate clinical characteristics, responses to therapy, and disability outcomes in patients with NMOSD. Methods: Patients diagnosed as NMOSD and following up for at least 1 year at a tertiary care center in India were recruited. Patient data were collected ambispectively from January 2012 until December 2018. Results: A total of 106 patients (29M/77F) with NMOSD were evaluated. The mean age of onset was 29 (±11.6) years. About 77 patients (72.64%) were positive for the AQP4 antibody. Age of onset was higher for those presenting with an opticospinal syndrome (34.2 years) as compared to either isolated longitudinally extensive transverse myelitis (LETM) (30 years) or optic neuritis (ON) (25.3 years). The most common syndrome at onset was LETM in 57 patients (53.77%) followed by ON in 31 patients (29.24%). Azathioprine was the most common immunotherapy (83.96%) prescribed followed by rituximab (7.54%) and mycophenolate mofetil (1.88%). There was a significant decrease in the number of relapses post-azathioprine (P < 0.001). Out of 67 patients with ON, 21 (31.34%) had complete recovery while 17 (25.37%) patients had a severe deficit at a 3-month follow-up. Out of 92 patients with a motor deficit, 49 (53.26%) patients had a partial motor deficit at a 6-month follow-up. The severe visual deficit at baseline and female gender predicted poor visual and motor recovery, respectively. Conclusion: This is the largest descriptive study on patients with NMOSD from India. Relapse rates were similar irrespective of the clinical presentation, age, gender, and disease course. Treatment with immunosuppressive treatment significantly affected the disease course. [ABSTRACT FROM AUTHOR]

In prior literature it was conjectured that the Indian stock market responses on domestic macroeconomic surprises are expected to be significantly influenced by global surprises. In this paper we empirically established that hypothesis. We used both the Event Analysis and VAR model. We found that global surprises consistently dominate Indian stock market and the influence of domestic macroeconomic surprises on it is relatively less. The understanding of stock market dynamics against domestic macroeconomic surprises and global factors can provide assistance to the policy makers for augmenting policy effectiveness and the corporate finance professionals for enhancing decision making. [ABSTRACT FROM AUTHOR]

Carbon credit is an element, which is used to aid in regulation of the amount of carbon dioxide that is being released into the air. This is the part of a global plan which has been created in an effort to reduce the global warming and its effect. This global plan works by capping the amount of total emissions that can be released by one organization. If there is a shortfall in the amount of gases that are used, there is a monetary value assigned to this shortfall and it may be traded and the trading of these carbon credits is known as Carbon Credits Trading. This research paper is focused on the Carbon Credits Trading Practices within Indian organizations. The main objective of this research paper is to explore the different carbon credits trading practices which are adopted by the Indian organizations. [ABSTRACT FROM AUTHOR]

PURPOSE: The information about the outcome of primary CNS lymphoma (PCNSL) in India is scarce, because there is no population-based or large hospital-based data. MATERIALS AND METHODS: This is a retrospective study that spanned 17 years (2001 to 2017) to study the outcome of PCNSL at the All India Institute of Medical Sciences (AIIMS), which is a tertiary care center in Northern India. RESULTS: Only one of 99 patients was positive for HIV serology. Diffuse large B-cell lymphoma was the most common histology (97.7%). The median patient age was 50 years (range, 13 to 70 years), and the ratio of men to women was 1.9. The median duration of symptoms before diagnosis was 3.5 months (range, 0.5 to 48 months), and 58.5% had a performance status (PS) of 3 or more. Multiple intracranial lesions were present in 81.8% of patients. Surgical resection was performed in 45%, and approximately 22% of patients were ineligible for treatment. Most patients (n = 73) were treated with high-dose methotrexate (HDMTX)–based regimens (ie, methotrexate, vincristine, and procarbazine with or without rituximab). Pharmacokinetic monitoring of methotrexate was not available at our center. HDMTX-related mortality was 3.9%. The median follow-up duration, event-free survival (EFS), and overall survival (OS) were 34 months, 20.4 months, and 31.7 months, respectively. Addition of rituximab (n = 27) to MVP resulted in a higher objective response rate (88.9% v 73.9% without rituximab; P =.12), complete remission (81.5% v 56.5%; P =.03), 2-year EFS (57.3% v 40.4%; P =.02), and 2-year OS (61.6% v 53.4%; P =.056). CONCLUSION: This is the largest study of PCNSL from India. The patients were immunocompetent and young but presented with a high-burden disease that precluded treatment in approximately 22%. The treatment with HDMTX appears safe without pharmacokinetic monitoring. The outcome is comparable to those observed in the West, and rituximab use showed additional benefit. There are notable barriers with respect to management of PCNSL in the real world, and efforts are required to improve the outcome more. [ABSTRACT FROM AUTHOR]

This study analyzes the sensitivity of a series of Indian stock indices for the astonishing component of monetary and macroeconomic policy with the data set from 1 April 2004 to 31 July 2016. The immediate impact is assessed with event analysis, and the dynamic effect is analyzed with the Vector Autoregression (VAR) model. The result of the event analysis indicates that the monetary policy surprise significantly affects the stock market and is more prominent than that of other macroeconomic surprises. Unlike the event study, the VAR analysis found that the other macroeconomic surprise also affects stock return. The study also highlights the industry effect and size effect, which is coherent with the predictions of the CAPM (Capital Asset Pricing Model) model. While many studies have been conducted on the monetary policy surprise in the developed economy, there are relatively few studies on macroeconomic surprises. Some studies conducted in India have analyzed the impact of monetary policy surprises on stock price; however, to the best of our knowledge, none of the studies has examined the simultaneous effect of both macroeconomic and monetary policy surprise. The study is relevant because the responses differ across sectors and vary with firm sizes. Thus, the study can effectively be used as a hedging instrument. Furthermore, the stock market acts as a vital channel for policy transmission and a critical decision driver for corporate finance. The understanding of firm and stock market dynamics against macroeconomic surprises can help policymakers in enhancing policy effectiveness and corporate finance professionals in improving decision-making. [ABSTRACT FROM AUTHOR]

Purpose – The purpose of this paper is to investigate the effect of Total Quality Management (TQM) principles on organizational performance in small and medium enterprises (SMEs) in Indian auto component sector. Design/methodology/approach – The paper is based on survey data collected from quality heads of 120 Auto component SMEs in India and uses correlation and regression analysis to test the relationships between quality management principles (QMPs) and organizational performance. Findings – The study provides evidence that application of TQM principles such as “Process Approach,” “Mutually Beneficial Supplier Relationship” and “Factual Approach to Decision-Making” has a positive influence on the performance of Indian auto component SMEs. Practical implications – This study presents a number of managerial implications, specifically for quality managers in Indian auto component SMEs. The focus of these organizations should be on strengthening the supply chain and operations. The quality managers need to align processes and resources based on factual approach to decision making for achieving higher organizational performance. Management of the organizations should also analyze why the soft aspects of TQM, namely, Involvement of People, Customer Focus and Leadership are not contributing to the organizational performance in Indian auto component SMEs. Originality/value – This paper identifies the key QMPs that can influence organizational performance in SMEs in the Indian auto component sector whereas most existing studies have focussed only on Tier-1 organization in this sector. Unlike previous studies on this subject, the present study highlights the significant impact of predominantly hard factors of TQM such as “Process Approach” and “Factual Approach to Decision-Making” with only one soft factor of TQM, namely, “Mutually Beneficial Supplier Relationship” being significant. [ABSTRACT FROM AUTHOR]

Objectives: To review clinical characteristics and response to immunomodulation therapy in autoimmune encephalitis presenting with status epilepticus (SE), epilepsy, and cognitive decline. Design: Observational, prospective case series. Setting: All India Institute of Medical Sciences, New Delhi, India. Materials and Methods: Prospective analysis of 15 patients, who presented with SE, epilepsy, cognitive decline, and other neurological symptoms with positive autoantibodies. Demographic and clinical characteristics were recorded. Brain magnetic resonance imaging (MRI), cerebrospinal-fluid analysis (CSF), and tumor screening were done periodically. Treatment received and responses (categorized as per patients and treating doctor's information) were noted. Results: There were 15 (males = 10) patients of autoimmune encephalitis. The mean age of presentation was 24 years (range: 2-64 years). The most common onset was subacute (64%) and four (29%) patients presented as SE. Predominant clinical presentations were seizures (100%) almost of every semiology. CSF was done in 10 patients; it was normal in 60%. Brain MRI was done in all patients, in six (40%) it was normal, six (40%) showed T2W and FLAIR hyperintensities in bilateral limbic areas. Antibodies found were the N-methyl-D-aspartate receptor antibody in seven (50%), voltage-gated potassium channel antibody in five (36%), two of antiglutamic acid decarboxylase, and one patient with double stranded DNA (dsDNA) antibodies. None showed evidence of malignancy. Patients received immunotherapy, either steroids, intravenous immunoglobulin, or both. Follow-up showed significant improvement in majority of cases, neither further seizures nor relapse in nine (67%) cases. One death occurred, due to delayed presentation. Conclusions: Uncommon but potentially reversible causes of SE, epilepsy, and cognitive decline may be immune-related and high index of suspicion will prevent missing the diagnosis. [ABSTRACT FROM AUTHOR]

Purpose: To assess the outcome of a pediatric population operated for drug-resistant epilepsy from a large tertiary care center in India. Methods: Retrospectively: quality of life (QOL); prospectively: preoperative assessment included interictal EEG, MRI (as per epilepsy protocol), video-EEG. Ictal SPECT (with subtraction) and PET were performed when required. QOL scores were assessed using the HASS or SSQ for seizure severity, Quality of Life in Childhood Epilepsy (QOLCE) for QOL, and Child Behavior Check List (CBCL) for behavior. Results: 142 were operated from January 2000 to June 2011 by the senior author. 118 patients with at least 1 year of follow-up were included in the study. Mean age at surgery was 9.8 ± 4.3 years. In addition, 40 patients underwent QOL assessment prospectively both before and after surgery. Mean duration of epilepsy was 5.3 ± 3.3 years. A class I outcome (Engel's) was seen in 79.5% patients, class II in 8.6%, class III in 10.7%, and class IV in 1 patient. As per surgical procedures, class I outcome in patients who underwent temporal resection, hemispherotomy and extratemporal resection was 76, 87 and 72%, respectively. QOL scores correlated with duration of seizures, epileptic encephalopathy and outcome of surgery, but not with side of surgery, age and sex. Conclusions: This study, the largest reported from India, has demonstrated satisfactory results for epilepsy surgery in children. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

SUMMARY We report three cases of spinal angiolipoma, two intramedullary and one epidural, and review their MRI features. Diagnoses were established preoperatively in all cases. Specific MRI features including the importance of postgadolinium fat-saturated T1-weighted images and presence of vascular flow-voids are discussed with histopathological correlation. A characteristic morphological appearance of intramedullary angiolipoma has been suggested. [ABSTRACT FROM AUTHOR]

• Rehabilitation related improvements in memory, anxiety and quality of life were sustained at 6 months. • Culturally tailored psychoeducation was beneficial. • Lack of understanding on the role of cognition in epilepsy is prevalent in patients and caregivers. • Neuropsychology referral should be made at the time of diagnosis. • Standard deviation cut-off may be an adequate index of meaningful change. Neuropsychological Rehabilitation (NR) helps manage cognitive deficits in epilepsy. As internationally developed programs have limited applicability to resource-limited countries, we developed a program to bridge this gap. This 6-week caregiver-assisted, culturally suitable program has components of (1) psychoeducation, (2) compensatory training, and, (3) cognitive retraining and is called EMPOWER (Indig e nized Ho m e Based Attention and Memory Rehabilitation P rogram f o r Adult Patients w ith Drug R e f r actory Epilepsy). Its efficacy needs to be determined. We carried out an open-label parallel randomized controlled trial. Adults aged 18–45 years with Drug Refractory Epilepsy (DRE), fluency in Hindi and or English, with impaired attention or memory (n = 28) were randomized to Intervention Group (IG) and Control Group (CG). The primary outcomes were objective memory (Auditory Verbal Learning Test), patient and caregiver reported everyday memory difficulties (Everyday Memory Questionnaire-Revised), number of memory aids in use, depression (Hamilton Depression Rating Scale), anxiety (Hamilton Anxiety Rating Scale) and quality of life (Quality of Life in Epilepsy-31). Intention to treat was carried out for group analysis. In the absence of norms necessary for computing Reliable Change Indices (RCIs), a cut-off of +1.0 Standard Deviation (SD) was utilized to identify clinically meaningful changes in the individual analysis of objective memory. A cut-off of 11.8 points was used for quality of life. Feedback and program evaluation responses were noted. The majority of the sample comprised DRE patients with temporal lobe epilepsy who had undergone epilepsy surgery. Group analysis indicated improved learning (p = 0.013), immediate recall (p = 0.001), delayed recall (p < 0.001), long-term retention (p = 0.031), patient-reported everyday memory (p < 0.001), caregiver-reported everyday memory (p < 0.001), anxiety (p = 0.039) and total quality of life (p < 0.001). Individual analysis showed improvement in 50 %, 64 %, 71 %, 57 %, and 64 % of patients on learning, immediate recall, delayed recall, long-term retention, and total quality of life respectively. Despite improvements, themes indicative of a lack of awareness and understanding of cognitive deficits were identified. Overall, the program was rated favorably by patients and caregivers alike. NR shows promise for patients with DRE, however larger studies are warranted. The role of cognition in epilepsy needs to be introduced at the time of diagnosis to help lay the foundation for education and acceptance. [ABSTRACT FROM AUTHOR]

Background Fragile X-associated Tremor/Ataxia syndrome (FXTAS) is a clinically heterogeneous disorder characterized predominantly by tremor, followed by late onset gait ataxia, autonomic dysfunction and/or cognitive impairment. We aimed to screen FMR1 -CGG repeats in our cohort of progressive late-onset cerebellar ataxia/tremor cohort to characterize the occurrence of FXTAS in India. Methods We have screened FMR1 -CGG repeats in 109 patients and 173 healthy control subjects. Our cohort comprised: a)group of patients with predominant cerebellar ataxia and/or tremor. b.)suspected cases of MSA and c.)patients who presented SCA12-like neurological manifestations (late onset predominant tremor and/or ataxia). All the cases were ruled out for known triplet-repeat-expansion (TRE) SCA mutations. Results We have found three FMR1 -premutation carriers among the cases. Two of them (with CGG-96 and CGG-102) were under evaluation for their SCA12-like manifestations and another (CGG-78) had progressive gait ataxia. Overall the frequency of FXTAS in our cohort was found to be 3.3% among cases of late onset cerebellar-ataxia/tremor; however, incidences were higher among cases with SCA12-like syndrome (9%, 2/23). Conclusion Finding FXTAS in patients with SCA12-like manifestation suggests that TRE in the 5′UTR of the gene is the common cue connecting two disorders with common phenotype of tremor/ataxia. This knowledge might shed light upon their sharing of molecular neuropathology. [ABSTRACT FROM AUTHOR]

Background: As the health systems around the world struggled to meet the challenges of COVID-19 pandemic, care of many non-COVID emergencies was affected., Aims: The present study examined differences in the diagnosis, evaluation and management of stroke patients during a defined period in the ongoing pandemic in 2020 when compared to a similar epoch in year 2019., Methods: The COVID stroke study group (CSSG) India, included 18 stroke centres spread across the country. Data was collected prospectively between February and July 2020 and retrospectively for the same period in 2019. Details of demographics, stroke evaluation, treatment, in-hospital and three months outcomes were collected and compared between these two time points., Results: A total of 2549 patients were seen in both study periods; 1237 patients (48.53%) in 2019 and 1312 (51.47%) in 2020. Although the overall number of stroke patients and rates of thrombolysis were comparable, a significant decline was observed in the month of April 2020, during the initial period of the pandemic and lockdown. Endovascular treatment reduced significantly and longer door to needle and CT to needle times were observed in 2020. Although mortality was higher in 2020, proportion of patients with good outcome were similar in both the study periods., Conclusions: Although stroke admissions and rates of thrombolysis were comparable, some work flow metrics were delayed, endovascular stroke treatment rates declined and mortality was higher during the pandemic study period. Reorganization of stroke treatment pathways during the pandemic has likely improved the stroke care delivery across the globe., (Copyright © 2021 Elsevier B.V. All rights reserved.)

The ongoing COVID-19 pandemic has forced every radiology set-up to evolve and formulate guidelines for day-to-day functioning. The sub-speciality of neuroradiology, both diagnostic and neuro-intervention, forms a very important part of any radiology or 'neuro-care' set-up. The present document is a consensus statement of the Indian Society of Neuroradiology, prepared after reviewing the available data and working experience. It scientifically tries to answer many questions faced by neuroradiologists everyday in practice. It encompasses simple things such as which patients need to be imaged, what precautions are essential, the work-flows, cleaning of radiology equipment, how to carry out neuro-interventions in COVID-suspect patients, and what procedures/tests to avoid, or their alternatives, to minimise the spread of COVID infection both to the patients and health care personnel. As radiology set-ups can be large, every sub-speciality may have certain precautions which will not be covered in general guidelines, and this document tries to answer those for neuroradiologists. Carefully evolved Standards of Operating Procedure (SOPs) and guidelines are the need of the hour to guide in providing uninterrupted and adequate services to the needy without compromising the safety of the specialised work force and facilities involved.

Background and Objectives: Ischemic stroke (IS) and coronary artery disease (CAD) share common risk factors and one may be the harbinger of the other. We aimed to study prevalence of symptomatic and asymptomatic CAD in a cohort of consecutive patients with IS and assess its relationship with intracranial and extracranial large artery cerebrovascular disease (LAD)., Methods: All consecutive eligible IS and Transient Ischemic Attack (TIA) patients were recruited into the study. Both clinically suspected and asymptomatic patients (N = 259) underwent myocardial Stress-rest Gated Technetium-99m (Tc99m) MIBI Myocardial Perfusion SPECT scan performed on a dual head SPECT-CT to estimate evidence of myocardial ischemia., Results: Three hundred patients completed the study. Forty one patients were previously diagnosed cases of definitive CAD. Twelve patients were clinically suspected to have CAD and 247 patients were asymptomatic. Among these, 12 patients (4.81%) had a positive SPECT. The overall prevalence of CAD was 17.67% (n = 53). Presence of diabetes was an independent predictor of CAD (OR 1.98, 95% CI 1.07-3.67. P .02). No significant association was found between the presence of LAD and CAD in all subgroup comparisons. However, there was a suggestion of higher LAD among patients with known CAD compared with others., Conclusions: CAD is prevalent in patients with ischemic stroke. No definitive relationship was found between CAD and intracranial or extracranial LAD. Population based stratification tools are needed to further assess the need to detect subclinical CAD in patients with stroke., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background & Objectives: Spinocerebellar ataxia 7 (SCA7) is a rare form of neurodegenerative disorder with the clinical manifestation of cerebellar ataxia and retinal degeneration. In this study we describe the clinico-genetic characteristics of nine SCA7 families of Indian origin and cross compare these with other available worldwide studies., Methods: Thirty five individuals from nine SCA7 families were clinico-genetically characterized and CAG repeat distribution analysis was carried out in 382 control DNA samples from healthy controls (derived from 21 diverse Indian populations based on ethnic and linguistic and geographical location)., Results: Of the nine families studied, 22 affected individuals and one asymptomatic carrier were identified. The average age at disease onset was 23.4±12.6 yr. The length of expanded CAG ranged from 40-94 with mean value of 53.2±13.9. The main clinical findings in affecteds individuals included cerebellar ataxia, and retinal degeneration along with hyper-reflexia (95%), slow saccades (85%) and spasticity (45%). Analysis of the association of number of CAG repeats with disease onset revealed that <49 repeats were associated with earlier age at onset in South East Asians compared to European populations. Further analysis of CAG repeats from 21 diverse Indian populations showed pre-mutable repeats (28-34) alleles in the IE-N-LP2 population. Six of the nine families identified in this study belonged to the same ethnic population., Interpretations & Conclusion: Our results show that presenece of SCA7 is relatively rare and confined to one ethnic group from Haryana region of India. We observed a homogeneous phenotypic expression of SCA7 mutation as described earlier and an earlier age of onset in our patients with CAG <49. The identification of pre-mutable allele in IE-N-LP2 suggests this population to be at the risk of SCA7.

Purpose: To assess the outcome of a pediatric population operated for drug-resistant epilepsy from a large tertiary care center in India., Methods: Retrospectively: quality of life (QOL); prospectively: preoperative assessment included interictal EEG, MRI (as per epilepsy protocol), video-EEG. Ictal SPECT (with subtraction) and PET were performed when required. QOL scores were assessed using the HASS or SSQ for seizure severity, Quality of Life in Childhood Epilepsy (QOLCE) for QOL, and Child Behavior Check List (CBCL) for behavior., Results: 142 were operated from January 2000 to June 2011 by the senior author. 118 patients with at least 1 year of follow-up were included in the study. Mean age at surgery was 9.8 ± 4.3 years. In addition, 40 patients underwent QOL assessment prospectively both before and after surgery. Mean duration of epilepsy was 5.3 ± 3.3 years. A class I outcome (Engel's) was seen in 79.5% patients, class II in 8.6%, class III in 10.7%, and class IV in 1 patient. As per surgical procedures, class I outcome in patients who underwent temporal resection, hemispherotomy and extratemporal resection was 76, 87 and 72%, respectively. QOL scores correlated with duration of seizures, epileptic encephalopathy and outcome of surgery, but not with side of surgery, age and sex., Conclusions: This study, the largest reported from India, has demonstrated satisfactory results for epilepsy surgery in children., (Copyright © 2011 S. Karger AG, Basel.)