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Highlights
A multipronged teleneurology based approach for management of infantile spasms is needed for developing countries.
Key principles: Fundamentals of management of IS, decentralization of patient care to local health providers, and early initiation of first-line therapeutic options.
Efforts should be made for improving sensitivity and specificity of diagnosis.
Constant motivation of parents and local health providers for monitoring therapeutic response, adverse effects of therapy, and infections.
Background: Children are most vulnerable to tubercular meningitis. Neuroimaging is an important initial investigation in tubercular meningitis. Objective: This study was done to describe the clinical profile, neuroimaging changes, and clinical outcome in children with tubercular meningitis. Methodology: This was an observational cohort study on children with tubercular meningitis, between January 2012 and June 2018. Tubercular meningitis was diagnosed on the basis of clinical criteria, cerebrospinal fluid analysis, neuroimaging, and response to antitubercular drug treatment. Preferably magnetic resonance imaging (MRI) with contrast was done. Result: Out of 79 pediatric patients, 17 patients were lost during follow-up; thus, a total of 62 patients were studied. Mean age at presentation was 7.040 (±3.99 SD) year, 51.6% children were male. Rural children were more affected. Twenty eight (45.2 patients had contact with a person with tuberculosis. Only 3 (4.8%) patients presented within 10 days of duration of illness. Most of the cases (67.7%) were in stage 2 at the time of diagnosis. The most common clinical feature was fever, seizure, and signs of meningeal irritation (all present in 12.9%). In neuroimaging most common findings were tuberculoma (50%), hydrocephalus (54.8%), and basal meningeal enhancement (33.8%). Regarding outcome, 6 (9.67%) patients expired and 47 (75%) patients had sequelae. The most common complications were hydrocephalus (30.64%) and intellectual disability (12.9 ). Hydrocephalus was the most common neuroimaging finding among the patients who expired (33%). Conclusion: Hydrocephalus is the most common neuroimaging finding. Normal neuroimaging is associated with good outcome whereas all the patients who died had abnormal neuroimaging.
Background and Purpose: In childhood epilepsy, genetic etiology is increasingly recognized in recent years with the advent of next generation sequencing. This has broadened the scope of precision medicine in intractable epilepsy, particularly epileptic encephalopathy (EE). Developmental disorder (DD) is an integral part of childhood uncontrolled epilepsy. This study was performed to investigate the genetic etiology of childhood epilepsy and DD.Methods: In this study, 40 children with epilepsy and DD with positive genetic mutation were included retrospectively. It was done in a tertiary care referral hospital of Bangladesh from January 2019 to December 2020. Genetic study was done by next generation sequencing. In all cases electroencephalography, neuroimaging was done and reviewed.Results: In total, 40 children were enrolled and the average age was 41.4±35.850 months with a male predominance (67.5%). Generalized seizure was the predominant type of seizure. Regarding the association, intellectual disability and attention deficit hyperactivity disorder was common. Seventeen cases had genetically identified early infantile EE and common mutations observed were SCN1A (3), SCN8A (2), SLC1A2 (2), KCNT1 (2), and etc. Five patients of progressive myoclonic epilepsy were diagnosed and the mutations identified were in KCTD7, MFSD8, and CLN6 genes. Three cases had mitochondrial gene mutation (MT-ND5, MT-CYB). Some rare syndromes like Gibbs syndrome, Kohlschütter-Tönz syndrome, Cockayne syndrome, Pitt-Hopkins syndrome and cerebral creatine deficiency were diagnosed.Conclusions: This is the first study from Bangladesh on genetics of epilepsy and DD. This will help to improve the understanding of genetics epilepsy of this region as well as contribute in administering precision medicine in these patients.
Objective: Autism is a neurodevelopmental disorder where genetic factors play causal role. This case-control study explored the association between perinatal and socioeconomic status with risk of autism.Method: It was a case control study among children who came from different areas of the country during the period of 2005 to 2015 for assessment in the outdoor facility of Bangabandhu Sheikh Mujib Medical University. One hundred children were diagnosed autism by Autism Diagnostic Observation Schedule (ADOS)-Generic. Among them 79 were boys and 21 were girls. Another one hundred children without autism were taken as control, 65 of them were boys and 35 were girls. Perinatal and socioeconomic risk factors were analyzed in these two groups, using medical records.Result: Mean age of the ASD children was 3.51+1.58 year and control was 3.80+1.53. Male predominance was observed in ASD children. After univariate analysis three significant variables identified to pose as risk for autism. These were parental advanced level of education (P=<0.001), urban residence (P=<0.001) and neonatal jaundice (P=<0.001). Variables like male sex, occupation as service holder and low birth weight at birth approached significance at the 0.05 level.
Conclusion:Results suggested that parental profile as well as perinatal environment are associated with risk of autism and this factor act independently.
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