Acquired Demyelination Syndrome in Children and Adolescents: 10 Years Experience from a Tertiary Care Centre in North India.

Background: The childhood central nervous system (CNS) acquired demyelinating syndromes (ADS) can be monophasic or recurrent, with both having considerable overlap in the first decade of life.

Objectives: The objective of the study was to describe clinical and radiological features, immunological characteristics, response to therapy and difference between monophasic and first episode of recurrent disorders of pediatric-onset CNS ADS.

Methods: Case records of all patients presenting with CNS ADS to the Department of Pediatrics between January 2009 to December 2018 were retrospectively reviewed.

Paroxysmal Sympathetic Hyperactivity in Childhood Tuberculous Meningitis: A New Association.

Background: We sought to estimate the prevalence and clinical characteristics of paroxysmal sympathetic hyperactivity (PSH) in childhood tuberculous meningitis.

Methods: Hospital records of children (6 months to 14 years) with tuberculous meningitis were retrospectively analyzed from September 2019 through January 2022. In September 2019, the first case of paroxysmal sympathetic hyperactivity in tuberculous meningitis was identified in our division.

Transmethylation and Oxidative Biomarkers in Children with Autism Spectrum Disorder: A Cross Sectional Study.

We aimed to investigate the potential role of biomarkers of transmethylation, oxidative stress, and mitochondrial dysfunction in children with Autism Spectrum Disorder (ASD) by comparing them with that of typically developing children (TDC) controls. We also tried to correlate them with severity of autism, sensory issues, behavioural comorbidities and developmental quotients 119 with ASD and 52 age and sex matched typically developing children (TDC) controls were enrolled excluding those with chronic-illness or on any antioxidant therapy/multivitamins/anti-epileptic drugs. Median levels of biomarkers - serum homocysteine, cysteine, methionine, urine uric acid-to-creatinine ratio, arterial lactate, serum vitamin E, vitamin B12, folate, Nε-carboxymethyllysine, Nω- carboxymethylarginine (CMA), dityrosine and MTHFR C677T polymorphism were calculated.

'Remote inhibition' of motor cortex in Epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS): A TMS based cortical excitability study.

Objectives: The study compared real-time motor cortex excitability using transcranial magnetic stimulation (TMS)-derived parameters between children with epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS) and age-matched neurotypical controls. The EE-SWAS group received steroids as standard of care and were longitudinally followed for three months.

Materials & Methods: Children aged 5-12 years with immunotherapy-naive EE-SWAS (spike-wave-index≥50 %) and neurotypical controls were enrolled.

Evaluation of Infantile Brachial Plexopathy Using 3T MRI and High-Resolution Ultrasound: Experience From a Tertiary Care Centre.

Background: Currently, clinical assessment is the main tool for the evaluation of brachial plexus injury, complemented by electrophysiologic studies (EPS), and imaging studies whenever available. Imaging plays an important role as it enables the differentiation of pre-ganglionic and postganglionic injuries, and adds objectivity to presurgical evaluation.

Objectives: The primary objective was to evaluate the utility of magnetic resonance imaging (MRI) and high-resolution ultrasonography (USG) in the localization and characterization of brachial plexus injury in infants.

NDUFV1-Related Mitochondrial Complex-1 Disorders: A Retrospective Case Series and Literature Review.

Background: Pathogenic variants in the NDUFV1 gene disrupt mitochondrial complex I, leading to neuroregression with leukoencephalopathy and basal ganglia involvement on neuroimaging. This study aims to provide a concise review on NDUFV1-related disorders while adding the largest cohort from a single center to the existing literature.

Methods: We retrospectively collected genetically proven cases of NDUFV1 pathogenic variants from our center over the last decade and explored reported instances in existing literature.

Disseminated phaeohyphomycosis due to Cladophialophora bantiana in an immunocompetent child.

A 3-year-old boy presented with acute headache, vomiting and right focal clonic seizures without history of fever, joint pain or altered sensorium. Neuroimaging showed multifocal contrast enhancing lesions with significant perilesional edema. CECT chest and abdomen showed multiple variable sized nodules in the lungs and hypodense lesion in liver with mesenteric lymphadenopathy.

Efficacy of daily versus intermittent low glycemic index therapy diet in children with drug-resistant epilepsy: A randomized controlled trial.

Introduction: The predominant reason for the discontinuation of low glycemic index therapy (LGIT) in children with epilepsy is the dietary restrictions imposed therein. This trial intended to compare the efficacy of daily and intermittent LGIT in children with drug-resistant epilepsy (DRE).

Methods: This study was performed between February 2018 and January 2019 to compare the efficacy of daily and intermittent LGIT in children aged 1-15 years with DRE following 24 weeks of dietary therapy.

Autoimmune Encephalitis as a Rare Paraneoplastic Syndrome in Adrenocortical Carcinoma.

Paraneoplastic neurologic syndromes (PNSs) are rare in pediatrics and are understood to be consequences of cross-reactivity against various neuroendocrine antigens expressed on cancer cells. Here, we report a case of autoimmune encephalitis, a type of PNS associated with a case of adrenocortical carcinoma (ACC), that had a clinical response to immunosuppressive therapy. ACC is a rare tumor with controversial tissue of origin but expresses various neuroendocrine antigens that could be the possible mechanism for this rare yet interesting association.

Child Neurology: Allgrove Syndrome: An Intriguing Etiology of Motor Neuron Disease in Children.

Motor neuron diseases are a rare group of neurodegenerative disorders with considerable phenotypic heterogeneity and a multitude of etiologies in the pediatric population. In this study, we report 2 unrelated adolescents (a boy and a girl) who presented with 4-6 years of progressive difficulty in walking, thinning of limbs, and gradually progressive darkening of the skin. Examination revealed generalized hyperpigmentation of skin and features suggestive of motor neuron involvement such as tongue atrophy, wasting of distal extremities, and brisk deep tendon reflexes.