Context: To evaluate the efficacy of diffusion fiber tractography (DFT) and voxel-based morphometry (VBM) for lateralizing language in comparison with functional magnetic resonance imaging (fMRI) to noninvasively assess hemispheric language lateralization in normal healthy volunteers. Aims: The aim of the present study is to evaluate the concordance of language lateralization obtained by diffusion tensor imaging (DTI) and VBM to fMRI, and thus to see whether there exists an anatomical correlate for language lateralization result obtained using fMRI. Settings and Design: This is an advanced neuroimaging study conducted in normal healthy volunteers. Subjects and Methods: Fifty-seven normal healthy subjects (39 males and 18 females; age range: 15-40 years) underwent language fMRI and 30 underwent direction DTI. fMRI language laterality index (LI), fiber tract asymmetry index (AI), and tract-based statistics of dorsal and ventral language pathways were calculated. The combined results were correlated with VBM-based volumetry of Heschl's gyrus (HG), planum temporale (PT), and insula for lateralization of language function. Statistical Analysis Used: A linear regression analysis was done to study the correlation between fMRI, DTI, and VBM measurements. Results: A good agreement was found between language fMRI LI and fiber tract AI, more specifically for arcuate fasciculus (ArcF) and inferior longitudinal fasciculus (ILF). The study demonstrated significant correlations (P < 0.05) between blood-oxygen-level dependent (BOLD) fMRI activations, tract-based statistics, and PT and HG volumetry for determining language lateralization. Conclusions: A strong one-to-one correlation between fMRI, laterality index, DTI tractography measures, and VBM-based volumetry measures for determining language lateralization exists.

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Background: Multiplex ligation-dependant probe amplification (MLPA) is a highly sensitive and rapid alternative to multiplex polymerase chain reaction (PCR). Muscle biopsy should be reserved for mutation-negative cases. Materials and Methods: An attempt was made to compare the sensitivity and pattern of mutations by mPCR and MLPA testing in a cohort with suspected Duchenne muscular dystrophy (DMD). Eighty-three children with DMD were enrolled for mPCR and MLPA testing. MLPA-negative cases underwent muscle immunohistochemistry (IHC) for dystrophin. Results: Mean age of onset was 45.3 ± 25.2 months; and mean duration of illness was - 53.3 ± 30.8 months. About 11.9% patients had delayed mental milestones. Mean creatine kinase (CK) value was 12136.1 ± 8591.1 LU/L. mPCR detected deletions in 60/83 (72.3%). Proximal deletions were found in 8 (8.6%), distal deletions in 51 (54.8%), and, both proximal and distal deletions were found in 1. Majority of the deletions were <5 exons [34(36.6%)]; two showed large deletions of >10 exons (2.2%). Deletions in hot spot region occurred in 83.3%. MLPA in the same 83 samples detected deletions in an additional six cases and duplications in 6 (6.5%). Combined detection rate of deletion was 79.5%. Duplications were found in 7.2% of the whole sample. MLPA showed 14 (15.1%) proximal and 57 (61.3%) distal deletions, and proximal and distal deletion in 1. Large deletions (>10 exons) were seen in 6.5%, and single deletions were observed in 24 (36.4%). Most common multiple exon deletion was seen at 45-52 region in 7 samples (10.6%). Longest duplication extended from exon 60 to 66. In the 11 MLPA-negative cases, IHC confirmed dystrophinopathy in 36.36%, sarcoglycanopathy in 36.36%, and no deficiency in 27.27%. Conclusions: This is the first study from India and possibly in English literature, comparing the sensitivity and pattern of mutations by both mPCR and MLPA in the same cohort of DMD. It further validates that 36.4% of MLPA-negative cases were confirmed to have DMD by IHC. The clinical accuracy has been very high in our cohort. MLPA-negative samples should be subjected for next-generation sequencing before contemplating a biopsy.

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Aims: Existing scales for functional grading of patients with cervical spondylotic myelopathy (CSM), such as the Nurick scale and modified Japanese Orthopedic Association (mJOA) scale, do not address certain culture-specific activities of the Indian population while grading patients with CSM. Materials and Methods: We modified the Nurick scale and mJOA scale to develop the Indian modifications of Nurick (imNurick) and mJOA scales (imJOA and imJOA scales), respectively, and then evaluated these modified scales in 93 patients with CSM to determine whether these modifications had a meaningful impact on the functional scores of these patients. Results: There was good interobserver agreement in the assessments documented in all the four scales (Nurick grade, imNurick grade, mJOA scale, and imJOA scale) (kappa = 1). Both Nurick grading (z = 4.4, P = 0.00) and imNurick grading (z = 5.5, P = 0.00) had a valid construct when tested against lower limb mJOA (llmJOA) score. The Indian modified upper limb JOA (imulmJOA) score too had a good construct with modified upper limb JOA (ulmJOA) score (z = 2.5, P = 0.01). There was substantial agreement between Nurick grade and imNurick grade (weighted kappa of 0.75) when taken as a whole group and between ulmJOA score and imulmJOA scores (weighted kappa of 0.75). However, there was significant disagreement between the Nurick grade and imNurick grade scales in patients who were Nurick grade 2 and 3 (kappa = 0.07). Conclusions: The proposed Indian modifications of Nurick grade and mJOA scale that incorporate the ethnic practices of the Indian population and some Asian population are better discriminators of different levels of functional ability among patients with CSM in this population, as compared to the existing Nurick grading and mJOA scale.

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A rare case of cavernoma in the region of the septum pellucidum is reported. A 35-year-old female patient presented with chronic headaches. Her neurological exam was normal. Her magnetic resonance (MR) imaging showed a lesion within the inferior aspect of the septum pellucidum extending into the anterior third ventricular region, blocking the foramen of Monro, resulting in moderate supratentorial asymmetrical hydrocephalus. A central neurocytoma or subependymoma was suspected on imaging. Complete excision of the septum pellucidum cavernoma was performed using microneurosurgical techniques through an interhemispheric transcallosal route. The patient had an excellent outcome and is cured. Although rare, septum pellucidum cavernomas should be considered in the differential diagnosis of anterior third ventricular lesions in the region of foramen of Monro. The unusual location, atypical radiological features, differential diagnosis as well as surgical nuances in the management of a cavernoma in the septum pellucidum and anterior third ventricular region are discussed in the light of current literature.

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Background: Primary central nervous system lymphomas (PCNSL) constitute a rare group of extranodal non-Hodgkin's lymphomas (NHLs). Aim: To study the clinical and immunophenotypic profile of patients with a PCNSL who presented between the years 2000 and 2013 in a tertiary care center in South India. Materials and Methods: This was a retrospective study. Demographic and clinical data were obtained from the clinical case records. Inclusion criteria: Cases of PCNSL involving brain. Exclusion criteria: Cases of PCNSL involving the spinal cord, meninges and orbit as well as intravascular large B-cell lymphoma, lymphomas with evidence of systemic disease or secondary lymphomas. Archived slides and tissue blocks were retrieved. All cases had hematoxylin and eosin stained sections and immunohistochemistry for CD20, CD3, and MIB-1. Additional immunohistochemistry was performed for CD10, BCL6, and MUM1 on paraffin blocks with sufficient tissue. Results: There were a total of 73 cases with the mean age of presentation being 45.9 years (range 8-71 years) and with a male predominance (male: female (M:F) = 2.3:1). Headache was the commonest presenting complaint. The mean duration of symptoms was 10.6 weeks. All patients were immunocompetent. Most tumors were supratentorial in location. Out of 73 cases, 70 presented with a diffuse large B-cell lymphoma (DLBCL), two with a Burkitt's lymphoma, and one with a lymphomatoid granulomatosis. Only 51 of the DLBCL cases had sufficient tissue for additional studies. Non-germinal center was the most common phenotype seen in 65.7% (33/51) of cases. Germinal center B-cell (GCB) phenotype was seen in 18/51 cases (34.3%). Conclusion: DLBCL constituted the majority of PCNSLs and although non-germinal center was the predominant phenotype, more than a third of the cases were of the GCB phenotype. As the germinal center phenotype is known to have a better prognosis, further studies to explore its relevance in the Asian population are indicated.

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Background: The Montreal Cognitive Assessment is a brief and easy screening tool for accurately testing cognitive dysfunction in Parkinson's disease. We tested its validity for use in non-English (Malayalam) speaking patients with Parkinson's disease. Materials and Methods: We developed a Malayalam (a south-Indian language) version of Montreal Cognitive Assessment and applied to 70 patients with Parkinson's disease and 60 age- and education-matched healthy controls. Metric properties were assessed, and the scores were compared with the performance in validated Malayalam versions of Mini Mental Status Examination and Addenbrooke's Cognitive Examination. Results: The Montreal Cognitive Assessment-Malayalam showed good internal consistency and test-retest reliability and its scores correlated with Mini Mental Status Examination (patients: R = 0.70; P < 0.001; healthy controls: R = 0.26; P = 0.04) and Addenbrooke's Cognitive Examination (patients: R = 0.8; P < 0.001; healthy controls: R = 0.52; P < 0.001) scores. Conclusion: This study establishes the reliability of cross-cultural adaptation of Montreal Cognitive Assessment for assessing cognition in Malayalam-speaking Parkinson's disease patients for early screening and potential future interventions for cognitive dysfunction.

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Introduction : Odontoid fractures constitute 9-20% of all adult cervical spine fractures. The present study was carried out to focus on the nuances involved in the surgical management of odontoid fractures. Materials and Methods: Patients with an odontoid fracture, admitted for surgical stabilization, between January 2008 and March 2014, were included in the study. Results: Among 142 patients [127 male and 15 female patients; median age: 28 years range 4-75 years], type II odontoid fractures were present in 111, type IIA fractures in 8, and type III fractures in 23 patients. 58.5% patients had been involved in a motor vehicular accident while 38.7% had sustained a fall. Eighty-five patients (59.9%) with a well-reduced fracture and an intact transverse ligament underwent anterior odontoid screw (OS) placement; the other 57 patients (40.1%) underwent posterior fixation (PF). The mean follow-up duration was 22 months (range: 6 months-5.4 years). OS placement was successful in 82 patients (96.5%) with a fusion rate of 95% (95.8% in type II, 100% in type III, and 75% in type IIA odontoid fractures). The procedure-related morbidity was 11.7%. One patient died of sub-arachnoid hemorrhage (SAH) that occurred during OS placement. The PF procedures had a better fusion rate (96.5%). The latter patients, however, had significant restriction of their neck movements and an overall morbidity of 8.7%. The revision surgery rates after OS placement and PF fixation were 7% and 3.5%, respectively. Conclusions: Anterior OS fixation shows excellent fusion rates and should be the first-line management in reduced/non-displaced acute type II (including type IIA) and high type III odontoid fractures as it preserves cervical motion. PF, that has also been associated with an excellent fusion rate, should be reserved for patients where OS fixation has either failed or has not been feasible.

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In the last two decades, applications of deep brain stimulation (DBS) have expanded rapidly in the field of neurosciences. The most common indications for DBS are Parkinson's disease, medically refractory seizures, essential tremors, and primary dystonia. This device has also been used as an investigational tool in patients having Tourette's syndrome, tardive dyskinesia, and refractory seizures. In the field of psychiatry, DBS has been used for the treatment of refractory obsessive compulsive disorder and depression. The complications are mainly related to surgery, the device, and its stimulation. This article provides an overview of the current status and recent advances in the field of DBS.

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Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing. The features were of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. She was pulsed with intravenous immunoglobulin, methylprednisolone, and cyclophosphamide and showed a good improvement. In the absence of widespread inflammatory response and classical histopathology findings, it is important to diagnose this condition as it shows a good response to aggressive and prolonged immunotherapy.

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Glomus jugulare tumors are benign but locally aggressive tumors. Their location, spread to adjacent areas and biological behavior have been extensively studied and reported. However, controversy exists regarding the role of surgery and stereotactic radiosurgery. The optimal plan of management of the tumor in close proximity to the facial and lower cranial nerves, the internal carotid and vertebral arteries, the venous sinuses, and the neuraxis is still nebulous. This review will discuss the differing viewpoints and attempts to propose a rational strategy in dealing with these tumors

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Background: Beta-adrenergic antagonists have demonstrated beneficial effects in tumor progression and survivability in patients with various cancers by inhibiting norepinephrine-induced tumor cell migration. However, little is known about their effects on the outcomes of metastatic brain tumors (MBTs). This study was undertaken to evaluate the effects of beta-blockers, if any, on the outcome of MBTs, and their possible role in controlling tumor progression and survivability. Materials and Methods: A retrospective cohort analysis of 225 patients identified as having MBTs presenting to our institution from 2001 through 2013 was conducted by reviewing electronic patient records. Patients were categorized into three groups: Group A comprised hypertensives on beta-blockers only (40, 18%), Group B comprised hypertensive patients on antihypertensive medications other than beta-agonists (65, 29%), and Group C comprised normotensives (120, 53%). All outcomes were compared using the data on pre - and post-gamma knife radiosurgery (GKRS) for these groups. One-way analysis of variance (ANOVA) was used to compare the radiological and clinical outcomes in the patient population following beta-blockers usage in Group A versus groups B and C. Cox regression model was used to demonstrate prognostic factors for the outcome in patients having different primaries. Overall survival period was plotted on Kaplan-Meier curves. The log-rank (Mantel-Cox) test was used to analyze the survival difference in the cases. P < 0.05 was considered significant. Results: The mean age of patients was 57.34 ± 10.98 years (range: 30-87 years) and 44% were males. More than half (130/225, 58%) of patients with MBT had their primary tumor source in the lung, 16% in the breast, and 7% each in the kidneys and the rectum. Frontal lobe was the most commonly affected (80, 35.5%). Statistically significant control of tumor growth (P = 0.001), tumor progression (P = 0.0001), and higher survival outcomes (P = 0.015) were observed in Group A as compared to other groups. In comparing the different groups, breast primaries showed the strongest correlation to survival benefit (P = 0.049) from beta-blocker usage as a primary antihypertensive medication. Conclusion: Concomitant use of beta-blockers with conventional therapy may offer potential benefit to hypertensive patients developing MBTs by ameliorating tumor progression and conferring a survival advantage. This effect was most notable in patients with primary tumors originating in the breast. Prospective studies, molecular research, and randomized controlled trials are warranted to further explore this promising effect.

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Background: Paraneoplastic vasculitic neuropathy (PVN) is a rare paraneoplastic syndrome. It is characterized by non-systemic subacute vasculitic neuropathy. It is most commonly associated with small cell lung cancers (SCLC) and lymphomas. PVN presents as a painful symmetrical or asymmetrical sensorimotor axonal neuropathy. The neurological symptoms may predate the tumor and may be the initial manifestations, or they may develop after a tumor is diagnosed. Recognition of this entity is important because of its potential treatability. Aim: To study the clinical features of PVN and briefly review the literature. Materials and Methods: The data was collected retrospectively from the medical records of our hospital. Results: Of the 14 cases of paraneoplastic neuropathies, 4 had a PVN. The age of onset was more than 50 years and there was no sex preponderance. Pain was seen in three patients. Two patients were previously treated for a thymoma. Two patients, following their presentation with PVN, were diagnosed with a colonic carcinoma and lung carcinoma, respectively. Conclusions: The recognition of PVN is important as this syndrome may respond to immunosuppression and tumor removal.

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Background: Valproate is a commonly used anticonvulsant drug. Uridine 5΄-diphospho (UDP)-glucuronosyltransferase (UGT) contributes to around 50% of valproate metabolism and its polymorphisms may be important for explaining the considerable variation in valproate levels in patients with epilepsy. Aim: This study was aimed to analyze the genetic polymorphisms of UGT1A6 in Indian children with epilepsy and their potential influence on the pharmacokinetics of valproate. Setting and Design: This cross-sectional study was carried out in the Department of Pediatrics, All India Institutes of Medical Sciences (AIIMS), New Delhi, between March 2011 and July 2012. Materials and Methods: Children aged 3-12 years diagnosed with epilepsy on valproate monotherapy for at least 1 month were enrolled. They underwent a detailed clinical examination. The UGT1A6 polymorphisms were detected by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Random samples were checked by genetic sequencing. The steady-state plasma concentrations of valproate were measured by High Performance Liquid Chromatography (HPLC) and associated with UGT1A6 polymorphisms. Results: A total of 80 children were studied. The prevalence of UGT1A6 T19G was as follows: TT (45%), TG (38.8%), and GG (16.3%); that of UGT1A6 A541G was: AA (48.8%), AG (38.8%), and GG (12.5%); and that of UGT1A6 A552C was: AA (43.8%), AC (40%), and CC (16.3%). The association between valproate doses or standardized serum valproate concentration and the various UGT1A6 genotypes could not be studied reliably in this small study population. Conclusions: The frequencies of UGT1A6 geneotypes and alleles were reported in the study population.

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