Background: Subacute sclerosing panencephalitis is a progressive devastating condition due to persistence of mutant measles virus, affecting children and adolescents, characterised by myoclonus, seizures, and neuropsychiatric issues. Movement disorders apart from myoclonus are reportedly uncommon. We aimed to describe frequency and proportion of movement disorders among children with subacute sclerosing panencephalitis, hypothesizing that these occur more frequently than previously reported. Methods: In this cross-sectional study, we enrolled children with subacute sclerosing panencephalitis between 1 month and 18 years of age who fulfilled the diagnosis of subacute sclerosing panencephalitis as per modified Dyken criteria, and examined them for movement disorders. We also assessed their clinical profile and disease severity via Jabbour staging and modified Rankin Scale score. We compared demographic, clinical, and laboratory features of children with and without movement disorders. Results: We enrolled 50 children (36 males; 72%) (age range 1.5-14 years). Of these, 28 (56%) had movement disorders. Among movement disorders, the most frequent was myoclonus (92%), followed by ataxia (9; 18%), chorea-athetosis (7; 14%), dystonia (6; 12%), tremor (4; 8%), repetitive behavior (4; 8%), and parkinsonism (3; 6%). Movement disorders were the presenting feature of subacute sclerosing panencephalitis among 7 children. There were no significant differences in clinical or laboratory features among children with and without movement disorders. Conclusions: Movement disorders were frequent in subacute sclerosing panencephalitis. Hyperkinetic disorders were dominant. Dystonia and chorea-athetosis occurred more commonly among nonmyoclonus movement disorders. Movement disorders may manifest even in earlier stages of subacute sclerosing panencephalitis and may be the heralding feature. Recognition of these features is important to plan management and reduce morbidity.

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Background: Sepsis is a state of systemic inflammation due to an infectious etiology that may lead to multisystem dysfunction, hemodynamic instability, and even death. It has been postulated that there may be an underlying immunomodulatory process resulting from rapid and exaggerated activation of macrophages that results in a cytokine storm and the development of macrophage activation syndrome (MAS). Adding immunomodulation to standard therapy (antibiotics and supportive care) can improve the prognosis. Clinical Description: We present a series of three young children who presented with the clinical features of sepsis. All three showed poor clinical response to management with timely antibiotics and supportive care, even after 48–72 h of initiation. In addition, there was the development of thrombocytopenia and transaminitis. The suspicion of MAS prompted us to order ferritin, triglyceride, and fibrinogen levels and applies the 2016 diagnostic criteria for MAS. These were satisfied, thus establishing the diagnosis. Management: In all three cases, immunomodulatory agents (intravenous immunoglobulin with or without pulses of methylprednisolone) were added, after which there was a clinical improvement, normalization of biomarkers, and complete recovery. Conclusion: Early immunomodulatory therapy, in addition to antibiotics, is beneficial in the successful treatment of children presenting with sepsis-induced MAS, thus preventing further morbidity and mortality and improving outcomes.

Purpose: Nearly 25% to 30% of children with epilepsy develop drug-resistant epilepsy. Etiology of epilepsy, including drug-resistant epilepsy, varies with geographical region. Identifying paucity of etiologic data on drug-resistant epilepsy from our region and similar low-resource settings, we aimed to describe the clinical and etiologic profile of children and adolescents with drug-resistant epilepsy, to better inform region-specific concerns. Methods: A chart-based retrospective review covering 10 years (January 2011–December 2020) was conducted. Participants between 1 months and 18 years of age who fulfilled International League Against Epilepsy (ILAE) definition of drug-resistant epilepsy were enrolled. Clinical details, perinatal history, electroencephalography (EEG), magnetic resonance imaging (MRI), and other evaluation-based data were analyzed. Results: Five hundred ninety-three children (52.3% males) were enrolled. The median age at presentation was 63 (interquartile range [IQR] 12-72) months and median age at onset was 12 (IQR 2-18) months. The most frequent seizure type was generalized (76.6%). Of these, epileptic spasms (48.1%) were most frequent. Focal seizures comprised 22.9%. The predominant contributor to etiology was perinatal adverse events, including perinatal asphyxia (37.9%), neonatal hypoglycemic brain injury (15.6%), and neonatal sepsis/meningitis. Electroclinical syndromes were observed in 361 (60.9%) children. Of these, the most frequent were West syndrome (48%) and Lennox-Gastaut syndrome (6.2%). Conclusion: Perinatal brain injury and brain infections were the most common causes of drug-resistant epilepsy identified. These findings indicate an opportunity for reducing the burden of pediatric drug-resistant epilepsy in our region by instituting preventive measures, including improved perinatal care, promotion of institutional deliveries, optimized obstetric and neonatal care, and immunization for vaccine-preventable infections such as bacterial meningitis and Japanese B encephalitis.