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Objective: To study impact of COVID-19 pandemic on frequency, clinical/electrophysiological profile and treatment outcomes in pediatric Guillain-Barré syndrome (GBS). Background: GBS is the most frequent cause of pediatric acute flaccid paralysis. The effect of the COVID-19 pandemic on pediatric GBS is unclear in the literature. Methods: We conducted an ambispective, multicentric, cohort study involving 12 of 27 centres in GBS Consortium, during two periods: pre-COVID-19 (March-August 2019) and during COVID-19 (March-August 2020). Children ≤12 years who satisfied National Institute of Neurological Diseases and Stroke criteria for GBS/variants were enrolled. Details pertaining to clinical/laboratory parameters, treatment and outcomes (modified Rankin Scale (mRS) at discharge, GBS Disability score at discharge and 3 months) were analysed. Results: We enrolled 33 children in 2019 and 10 in 2020. Children in 2020 were older (median 10.4 [interquartile range 6.75–11.25] years versus 5 (2.5–8.4) years; P = 0.022) and had more sensory symptoms (50% versus 18.2%; P = 0.043). The 2020 group had relatively favourable mRS at discharge (median 1 (1–3.5) versus 3 (2–4); P = 0.042) and GBS disability score at 3 months (median 0 (0–0.75) versus 2 (0–3); P = 0.009) compared to 2019. Multivariate analysis revealed bowel involvement (P = 0.000) and ventilatory support (P = 0.001) as independent predictors of disability. No child in 2020 had preceding/concurrent SARS-CoV2 infection. Conclusions: The COVID-19 pandemic led to a marked decline in pediatric GBS presenting to hospitals. Antecedent illnesses, clinical and electrophysiological profile of GBS remained largely unchanged from the pre-pandemic era.

Introduction/Aims: Studies conducted during the coronavirus disease 2019 (COVID-19) pandemic have reported varied data regarding the incidence of Guillain–Barre syndrome (GBS). The present study investigated demographic and clinical features, management, and outcomes of patients with GBS during a specified period of the COVID-19 pandemic, and compared these features to those of GBS in the previous year. Methods: A multicenter, ambispective cohort study including 26 centers across India was conducted. Data from a pre-COVID-19 period (March 1 to August 31, 2019) were collected retrospectively and collected ambispectively for a specified COVID-19 period (March 1 to August 31, 2020). The study was registered with the Clinical Trial Registry India (CTRI/2020/11/029143). Results: Data from 555 patients were included for analysis: pre-COVID-19 (n = 334) and COVID-19 (n = 221). Males were more commonly affected during both periods (male:female, 2:1). Gastroenteritis was the most frequent antecedent event in 2019 (17.4%), whereas fever was the most common event in 2020 (10.7%). Paraparesis (21.3% versus [vs.] 9.3%, P = 0.001) and sensory involvement (51.1% vs. 41.3%; P = 0.023) were more common during COVID-19 in 2020, whereas back pain (26.3% vs. 18.4%; P = 0.032) and bowel symptoms (20.7% vs. 13.7%; P = 0.024) were more frequent in the pre-COVID period. There was no difference in clinical outcomes between the two groups in terms of GBS disability score at discharge and 3 months after discharge. Independent predictors of disability in the pre-COVID period included areflexia/hyporeflexia, the requirementfor intubation, and time to bulbar weakness; in the COVID-19 period, independent predictors included time from onset to admission, intubation, and intubation requirement. The mortality rate was 2.3% during the entire study period (13/555 cases). Discussion: Results of this study revealed an overall reduction in the frequency of GBS during the pandemic. The lockdown likely reduced the risk for antecedent infections due to social distancing and improved hygiene, which may have resulted in the reduction of the frequency of GBS.

Aim: To study the clinical presentation, radiological findings, and therapy responsiveness of patients with biopsy-proven immunoglobulin G4 (IgG4)-related neurological disease. Methods: The study was conducted between January 2016 and March 2018 from the Department of Neurology and Pathology of Nizam's Institute of Medical Sciences. Patients with neurological symptoms and biopsy suggestive of IgG4-related disease (IgG4-RD) were included. These patients were studied for their demographic pattern and clinical presentation. The presence of serological markers such as vasculitic profile and IgG4 levels was analyzed. Radiological findings were studied in detail. Therapeutic agents used and the response to therapy were assessed. Results: There were six cases with IgG4-related neurological disease which were all hypertrophic pachymeningitis. The age ranged from 35 to 64 (mean = 46) years. The clinical presentation was acute in one, subacute in two, and chronic in three patients. The most common presenting symptom was headache (4), followed by gait and/or urinary disturbances (2), paraparesis (1), and diplopia (1). IgG4 levels were elevated in 50% of them. Pseudotumor-like mass and sinovenous thrombosis, not described previously, were seen in one patient. All the patients were treated with oral or intravenous steroid. Rituximab was given in three patients; azathioprine was the steroid-sparing agent in one patient. Those with acute/subacute onset of presentation had an excellent response to steroids. All the patients with a chronic duration of their symptoms received empirical anti-tuberculous therapy before a definitive diagnosis of Ig G4-RD was made. Conclusions: The characterization of patients with IgG4-related neurological disease based on the understanding of the clinical spectrum increases the confidence in the clinician to resort to early immunosuppression, thereby having prognostic implications.

Objective: Vasculitic neuropathy can be either restricted to the peripheral nerves or associated with systemic involvement of other organs. The objective of this study was to analyze the nerve biopsies reported as “vasculitic neuropathy” with clinical features. Materials and Methods: All cases diagnosed with vasculitic neuropathy were retrospectively analyzed and categorized as systemic vasculitis and nonsystemic vasculitic neuropathy based on the clinical features. The histological features were further evaluated and classified according to the Peripheral Nerve Society Guidelines. Results: Of the 126 cases, there were 65 nonsystemic vasculitis, 45 secondary systemic vasculitis, and 16 primary systemic vasculitis. Definite vasculitis was more common in the systemic vasculitis group. The epineurial vessels were predominantly involved with chronic axonal changes. Conclusion: The sensitivity of definite vasculitis on nerve biopsy was 54.76%. The sensitivity increases when the diagnostic criteria of definite and probable vasculitis were applied taking into account perivascular inflammation accompanied by vascular changes and axonopathy.

Toxoplasma is an obligate intracellular parasite that remains asymptomatic in humans but, at times, can cause devastating disease. Here, we describe an autopsy study of a young immunocompetent gentleman with no comorbidities whose presentation was acute transverse myelitis. Magnetic resonance imaging spine showed longitudinally extensive spinal cord lesion (LESCL) that mimicked neuromyelitis optica with normal brain imaging at presentation. Investigations showed albuminocytological dissociation which prompted a course of parenteral steroid. However, the lesion relentlessly progressed to involve the brain stem and cerebrum leading to toxoplasmic encephalitis that terminated fatally. This report highlights that toxoplasma can present as LESCL and needs to be considered in the differential diagnosis of atypical myelitis.

Author(s): Sireesha Yareeda [1]; Jabeen S. Afshan [1]; Mathukumalli L. Neeharika [1]; Rukmini M. Kandadai [1]; Suryaprabha P. Turaga [1]; Rupam Borgohain [1] Background Pediatric multiple sclerosis (POMS) is MS [...], Objective: The prevalence of pediatric onset multiple sclerosis (POMS) is 0.69-26.92 per 100,000 individuals. Here we describe the clinical profile and follow-up of patients with POMS from India where the data are scarce. Materials and Methods: This is a retrospective observational study; the data were collected from the ongoing MS registry from 2007. The clinical and demographic profile was studied and therapy response was documented. Results: There were nine patients with POMS with a mean age of 16.8 years (13-19). The lag in the diagnosis ranged from 0 to 8 years (mean of 2.5 years). Six had relapsing remitting variant of MS (RRMS). Optic neuritis was the most common presentation (44%). Oligoclonal bands were positive in 5 (55%). Conclusion: Optic neuritis was the common symptom followed by ataxia due to brainstem or spinal cord involvement. The lag period was long and the impairment in academic performance and social functioning was a concern. Keywords: Lag period, optic neuritis, pediatric multiple sclerosis

The article presents a case study of an elderly couple who experienced acute vision loss following accidental ingestion of closantel, a veterinary drug. Despite limited literature on closantel toxicity in humans, the study emphasizes the importance of early treatment with systemic corticosteroids and plasmapheresis to reverse toxic effects, highlighting the need for public awareness and appropriate drug labeling to prevent accidental toxicity.

Objective Electrical status epilepticus in sleep (ESES) is defined by near-continuous epileptiform discharges during sleep along with cognitive, behavioral, and/or imaging abnormalities. We studied the neurocognitive profile and their correlation with 18 F fluorodeoxyglucose positron emission tomography (FDG PET) brain abnormalities in children with ESES. Methods Fourteen children with ESES with normal magnetic resonance imaging (MRI) from March to December 2019 were included. The intelligence quotient (IQ) and child behavior checklist (CBCL) scores were estimated using validated scales, and FDG PET brain was done at the same point of time to look for cerebral metabolic defects which was compared with a control group. Results Fourteen patients with a mean age of 8.2 ± 2.7 years were analyzed. The average duration of epilepsy was 6 ± 2.8 years. The mean IQ was 72.4 ± 18.2 and mean CBCL score was 37.3 ± 11.8. There was negative correlation between IQ and CBCL (r = −0.55, p < 0.001). The duration of epilepsy also showed negative correlation with IQ (r = −4.75, p < 0.001). FDG PET scan showed predominant thalamic hypometabolism in 12 of 14 patients (85.7%) on visual analysis with multiple other hypometabolic cortical and subcortical regions in the brain. The quantitative analysis showed significant difference in metabolism of basal ganglion when compared with control group. The total number of hypometabolic regions seen in the brain showed moderate positive correlation with CBCL score but no significant correlation with the IQ of cases. Conclusion This study demonstrates functional impairment of cerebral cortical, basal ganglia, and thalamic hypometabolism in a cohort of ESES patients with normal structural MRI brain study. There was a moderate correlation of extent and pattern of cerebral hypometabolism with the neuropsychological status of the child and duration of epilepsy. [ABSTRACT FROM AUTHOR]

Hedgehog acyltransferase gene (HHAT)‐associated Nivelon‐Nivelon‐Mabile syndrome (NNMS) is a rare genetic disorder of multiple system involvement with microcephaly, central nervous system malformations, skeletal dysplasia, and 46,XY sex reversal. Other variable and inconsistent features reported in this disorder are muscle spasms, facial dysmorphism, prenatal onset growth restriction, microphthalmia, and holoprosencephaly. This is the sixth postnatal reported patient with biallelic variants in HHAT gene, who presented with microcephaly, short stature, muscle hypertrophy, muscle spasms, and facial dysmorphism. The most prominent and presenting finding in this patient were muscle hypertrophy and muscle spasms which had a clinical response to phenytoin and acetazolamide treatment. Our report emphasizes the phenotypic variability of NNMS and further reiterates muscle spasms as an important clinical manifestation of this extremely rare condition. [ABSTRACT FROM AUTHOR]

The article in Neurology India discusses an unusual case of a 24-year-old woman with a variant of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) that relapsed multiple times over 29 months. Despite initial treatments with IVIg and steroids, the patient experienced relapses during pregnancy and while on steroids. Eventually, a monthly IVIg regimen led to sustained remission with minimal disability. The case highlights the importance of individualized therapy for CIDP patients and the potential for successful treatment with low-dose maintenance IVIg. [Extracted from the article]

The document from Neurology India discusses the impact of Neuromyelitis Optica Spectrum Disorders (NMOSD) on pregnancy and the effects of pregnancy on NMOSD. It highlights the potential risks and considerations for patients with NMOSD who are pregnant, including the impact of medications on the baby and the disease's effects on fertility and relapse rates. The document also provides recommendations for managing NMOSD during pregnancy, such as treatment options for acute attacks and maintenance therapy, emphasizing the importance of careful planning and monitoring to ensure a safe pregnancy outcome. [Extracted from the article]

Background: Cerebellar ataxia is a disabling neurological symptom with extreme clinical and etiological heterogeneity.Objective: To study the clinical and molecular characteristics in patients with degenerative cerebellar ataxia.Materials and Methods: In this study, 150 South-Indian patients with degenerative cerebellar ataxia underwent a phenotype guided, sequential tiered testing. Phenotypic features studied included cerebellar symptoms, pyramidal and extrapyramidal features, and ophthalmic and systemic findings. Tier one included conventional tests such as short PCR/fragment analysis for spinocerebellar ataxia (SCA) subtypes 1, 2, 3, 6, 7, 8, 12, 17, and 36 and TP-PCR for Friedreich ataxia (FA). Tier two testing comprised next-generation sequencing (NGS)-based strategies reserved for select undiagnosed cases.Results: The clinical features were highly overlapping and had limited specificity, except in autosomal recessive ataxias and SCA 34. The overall diagnostic yield of our study was 49.3%. SCA 1, 2, and 3 were noted in 13 (12.6%), 12 (11.6%) and 14 (13.5%), respectively, out of the 103 tested, and FA was noted in 17/55 (30.9%) patients. SCA subtypes 6, 7, 8, 12, 17, and 36 were absent in the cohort studied. Targeted Sanger sequencing and NGS revealed some rare diagnoses in 17 among the 18 patients tested. Whole exome sequencing uncovered a novel genotype-phenotype association in a sibling-pair with ataxia, dysmorphism, and retinopathy.Conclusion: SCA 1, 2, 3 and FRDA were the most common causes of ataxia. SCA 6, 7, 8, 12, 17, and 36 were absent in the cohort studied. NGS testing revealed several rare forms of ataxia. Clinical features based testing is cost-effective, achieves good genotype-phenotype correlation, and prioritizes variants for further studies. [ABSTRACT FROM AUTHOR]

Introduction Chronic hyperglycemia is a key factor in the development of diabetic peripheral neuropathy (DPN), contributing significantly to the progression of this condition through the induction of oxidative stress. Elevated blood glucose levels lead to increased production of reactive oxygen species (ROS), which cause damage to neuronal cells and exacerbate neuropathic symptoms. Alpha-lipoic acid (ALA) is a potent antioxidant that neutralizes ROS and reduces oxidative damage. By addressing the mechanisms of neuropathy, ALA mitigates the effects of chronic hyperglycemia through antioxidant regeneration, inflammation reduction, and endothelial function improvement. As a result, ALA is emerging as a promising intervention for managing oxidative stress and inflammation in DPN. Methods Prospective, double-blind, placebo-controlled study, a total of 52 DPN subjects on gabapentin or pregabalin were randomly assigned to either ALA 600 mg oral once daily or placebo oral once daily for 12 weeks. Treatment outcome was assessed using vibration perception threshold (VPT), Neuropathy Total Symptom Score 6 (NTSS-6), quality of life (QOL) assessed by 12-Item Short Form Health Survey (SF-12) Questionnaire, oxidative stress biomarkers (malondialdehyde (MDA), nitric oxide (NO), and glutathione (GSH)) and inflammatory biomarker (high-sensitivity C-reactive protein (hs-CRP)) at baseline and every visit (four, eight, and 12 weeks). Neuron-specific enolase (NSE) levels were done at baseline and 12 weeks. Glycated hemoglobin (HbA1C) and safety parameters were done at screening and 12 weeks. Compliance with the study medication was assessed by pill count. Reported adverse drug reactions were recorded. Results A total of 52 subjects (males = 22; females = 30) with a mean age of 55.63 ± 7.5 years were randomized to receive either ALA or placebo. A significant reduction in VPT was observed with ALA at 12 weeks compared to baseline (from 26.92 ± 3.58 to 22.14 ± 1.94; p < 0.0001). Secondary parameters like NTSS-6, QOL by SF-12 questionnaire, NSE levels, oxidative biomarkers levels, and inflammatory biomarkers showed significant improvement with ALA compared to placebo. No serious adverse events were reported. Conclusion ALA demonstrated a protective effect against DPN by its antioxidant and anti-inflammatory effects and was found to have good safety., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. NIMS Institutional Ethics Committee issued approval EC/NIMS/2531/2020. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: This study was funded by the Indian Council of Medical Research (ICMR), New Delhi under the Product Development Centre(PDC). Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Pingali et al.)