Mapping cognitive dysfunction in relapsing multiple sclerosis with mild disability: A cross-sectional study from South India.

Background: Cognitive dysfunction in multiple sclerosis (MS) occurs early. Locally adapted neuropsychological data from India in MS is scarce.

Objectives: We aimed to identify the pattern of cognitive impairment in relapsing MS (RMS) with mild disability using a regionally-adapted MS-specific cognitive battery.

Clinical Spectrum of Biopsy Proven Mitochondrial Myopathy.

Objectives: Clinical spectrum of mitochondrial myopathy extends beyond chronic progressive external ophthalmoplegia (CPEO). While information on encephalomyopathies is abundant, clinical data on predominant myopathic presentation of mitochondrial disorders are lacking.

Materials And Methods: Clinical, electrophysiological, biochemical, and follow-up data of patients with predominant myopathic presentation and muscle biopsy confirmed primary mitochondrial myopathy was obtained.

World Psychiatric Association-Asian Journal of Psychiatry Commission on Psychiatric Education in the 21st century.

Psychiatric practice faces many challenges in the first quarter of 21st century. Society has transformed, as have training requirements and patient expectations, underlining an urgent need to look at educational programmes. Meanwhile, awareness has grown around psychiatric disorders and there are evolving workforce trends, with more women going to medical school and specialising in psychiatry.

Resting-State Functional Connectivity in Relapsing-Remitting Multiple Sclerosis with Mild Disability: A Data-Driven, Whole-Brain Multivoxel Pattern Analysis Study.

Multivoxel pattern analysis (MVPA) has emerged as a powerful unbiased approach for generating seed regions of interest (ROIs) in resting-state functional connectivity (RSFC) analysis in a data-driven manner. Studies exploring RSFC in multiple sclerosis have produced diverse and often incongruent results. The aim of the present study was to investigate RSFC differences between people with relapsing-remitting multiple sclerosis (RRMS) and healthy controls (HC).

Determinants of Suboptimal Outcome Following Thymectomy in Myasthenia Gravis.

Background: Response to thymectomy in myasthenia gravis (MG) is influenced by various patient-, disease-, and therapy-related factors.

Methods: Retrospective analysis of 128 patients with MG who underwent maximal thymectomy over 15 years was done to identify the determinants of suboptimal clinical outcome.

Results: Among the 128 patients, 62 (48.

Clinical and Genetic Profile of Autism Spectrum Disorder-Epilepsy (ASD-E) Phenotype: Two Sides of the Same Coin!

The clinical phenotype of autism spectrum disorder and epilepsy (ASD-E) is a common neurological presentation in various genetic disorders, irrespective of the underlying pathophysiological mechanisms. Here we describe the demographic and clinical profiles, coexistent neurological conditions, type of seizures, epilepsy syndrome, and EEG findings in 11 patients with ASD-E phenotype with proven genetic etiology. The commonest genetic abnormality noted was mutation (3), mutation (2), and 1p36 deletion (2).

Congenital myasthenic syndrome: Ten years clinical experience from a quaternary care south-Indian hospital.

Background: To ascertain the frequency, clinical spectrum and outcome of congenital myasthenic syndrome (CMS) patients who reported to the neuromuscular division of our quaternary medical center during the past ten years.

Methods: We performed a retrospective analysis of all the CMS patients who reported to us during the study period.

Results: Twenty-one patients of CMS attended our quaternary hospital over the past ten years.

Electroneurography and Advanced Neuroimaging Profile in Pediatric-onset Metachromatic Leukodystrophy.

Context: Metachromatic leukodystrophy (MLD) is a rare autosomal-recessive disorder characterized by demyelination of central and peripheral nervous system. There is scarcity of literature on the electrophysiological aspects of peripheral nerves and the advanced neuroimaging findings in MLD.

Aim: The aim was to study the nerve conduction parameters and advanced neuroimaging findings in patients with MLD.

Clinico-Genotypic Correlation: Recurrent Attacks of Paralysis and Skeletal Muscle SCN4A Mutation (p.Ile693Thr).

Skeletal sodium channel mutations have been known to demonstrate a multitude of clinical manifestations of which one such commonly known entity is paramyotonia congenita. We describe the clinical features of proband in our case report and the various phenotypic manifestations described with the mentioned mutation from different centres. Our case serves to highlight the heterogeneity that exists in SCN4A mutations and the possible effect of other genetic/environmental factors in determining the final phenotype.

An unusual presentation of scleromyxedema as inflammatory myopathy.

Scleromyxedema is a rare cutaneous mucinosis with frequent extracutaneous manifestations. Myopathy in scleromyxedema is a poorly recognized syndrome among neurologists and can mimic idiopathic and connective tissue disease-associated inflammatory myopathy. Diagnosis is suspected by the characterization of the skin lesions and clinched by skin and muscle biopsies.

Relapsing lumbosacral myeloradiculitis: An unusual presentation of MOG antibody disease.

Myelin oligodendrocyte glycoprotein antibody (MOG-Ab) seropositivity is being increasingly reported in diverse demyelinating syndromes with monophasic and relapsing presentations. Conus myelitis is described as a typical feature of MOG-Ab seropositivity. However, the association with lumbosacral radiculitis in this disease is not well-recognized.

Clinico-radiological correlation and surgical outcome of idiopathic spinal cord herniation: A single centre retrospective case series.

Idiopathic spinal cord herniation (ISCH) remains an obscure, under recognised but potentially treatable entity among the etiologies of compressive myelopathy. Here, we present a retrospective case series of eight patients of ISCH with their clinical features, imaging findings and long-term follow up data along with a relevant review of the literature. Mean age at presentation was 36 years with the duration of symptoms ranging from two months to more than thirty years.

Status epilepticus related to pregnancy: Devising a protocol for use in the intensive care unit.

Background: Status epilepticus (SE) related to pregnancy is rare and carries a significant risk to both the mother and the fetus.

Objectives: We conducted this study to devise a protocol for the management of SE related to pregnancy in a cohort of female patients admitted with SE during pregnancy.

Materials And Methods: All women who developed SE related to pregnancy (gestation, labor, and puerperium) between January 2000 and December 2016 were included.

An audit of the predictors of outcome in status epilepticus from a resource-poor country: a comparison with developed countries.

Status epilepticus is a neurological emergency with significant morbidity and mortality. This study describes the clinical profile, treatment, and predictors of outcome of status epilepticus in a tertiary referral centre in a developing country and aims to highlight the similarities and differences from data available from the western world. A retrospective analysis of data of patients treated for status epilepticus was conducted from prospectively maintained records, between January 2000 and September 2010.

A hospital-based registry of Creutzfeldt-Jakob disease: Can neuroimaging serve as a surrogate biomarker?

Aim: This study addresses the role of neuroimaging in addition to the available clinical criteria for Creutzfeldt-Jakob disease (CJD) and its impact on its diagnosis in the absence of cerebrospinal fluid (CSF) biomarkers and tissue-based approaches.

Methods: From a tertiary referral center in the city of Trivandrum, Kerala, South India, patients with rapidly progressive dementia (RPD) who fulfilled the World Health Organization (WHO) 1998 diagnostic criteria for CJD were included in this study. Their electrophysiological-clinical-radiological data were retrospectively studied and the results were analyzed.

Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis.

Objective: To elucidate the clinico-radiological features, treatment response and outcome of a large cohort of patients (n=20) with idiopathic hypertrophic pachymeningitis (IHP) and to examine if any of these features could differentiate between IHP and secondary causes of hypertrophic pachymeningitis (SHP).

Methods: 20 patients with IHP diagnosed between 1998 and 2009 formed the study cohort. We adopted a validated clinical score to quantitatively assess and document their neurological disability and to compare their pre- and post-treatment outcomes.

Multipoint incremental motor unit number estimation versus amyotrophic lateral sclerosis functional rating scale and the medical research council sum score as an outcome measure in amyotrophic lateral sclerosis.

Introduction: Monitoring the disease progression in amyotrophic lateral sclerosis (ALS) is a challenge due to different rates of progression between patients. Besides clinical methods to monitor disease progression, such as the ALS functional rating scale (ALSFRS) and the medical research council (MRC) sum score, quantitative methods like motor unit number estimation (MUNE) are of interest.

Objective: The objective of the present study is to evaluate the rate of progression in ALS using multipoint incremental MUNE and to compare MUNE, ALSFRS and MRC sum score at baseline and at 6 months for progression of the disease.