Comparison of clinical and laboratory profile of survivors and non-survivors of SARS-CoV-2-related multisystem inflammatory syndrome of childhood in India: An observational study.

AIM: The SARS-CoV-2 pandemic is characterised by multiple reports of paediatric multisystem inflammatory disease or multisystem inflammatory syndrome in children (MIS-C) with Kawasaki disease-like features often complicated by myocarditis, shock and macrophage activation syndrome. Certain clinical and laboratory markers may be used to identify high risk cases. METHODS: All sequentially admitted patients hospitalised between April 2020 and October 2020, who met the WHO case definition for MIS-C were included. Data included patient demographic information, presenting symptoms, organ dysfunction and laboratory parameters. SARS-CoV-2 infection was diagnosed by nasopharyngeal swab real-time reverse transcription-polymerase chain reaction and/or rapid antibody test for SARS-CoV-2 as recommended. The clinical and laboratory criteria were compared in the survival and non-survival groups. RESULTS: A total of 29 patients with MIS-C were treated during the study period. There were 21 survivors and 8 non-survivors. The non-survivors had more neurocognitive and respiratory symptoms along with increased incidence of myocarditis compared with survivors. The serum levels of CPK-MB, D-dimer, ferritin and triglyceride were significantly raised in non-survivors as compared to survivors. CONCLUSION: The non-survivor group had higher CPK and greater proportion of children with troponin-T elevation indicating higher incidence of myocardial injury and necrosis. The D-dimer, ferritin and triglyceride were also higher in the mortality group, indicating the greater extent of inflammatory damage in this group.

Promoting family integrated early child development (during first 1000 days) in urban slums of India (fine child 3-3-1000): Study protocol.

AIMS: This project tests a novel, targeted home visitation programme for child development targeted behaviour change during the first 1,000 days for families in Delhi urban slums. BACKGROUND: The first 1,000 days have highest brain development potential and is dependent on the available nutrition, health, social and cognitive stimulus. Over 1.3 million children are born annually in the slums of India and are at risk of limited development potential. The children in urban slums at multiplicity of adversities at family, society and environmental levels. No tools are available for the community health functionaries to support the families to promote child development. DESIGN: This cohort study targets provision of behaviour change interventions targeted at three groups (pregnant women, infants and children in year 2) to document the impact on child development. METHODS: This implementation project delivers nutrition, health and child stimulation integrated services for the families through existing government community health workers and nurses. These workers shall train the families using audio-visual messages in tablets and demonstration kits for practice through quarterly home visits. Data on health, nutrition and child development shall be collected at baseline, midterm and after one year. The data from these participants shall be compared with data from recently delivered women, children aged 13 months and 25 months without intervention to document the impact. DISCUSSION: The successful implementation of the project has potential for future integration of the child development components into the existing programme at scale. The learning from this project shall be useful for India and other developing countries. IMPACT: The first 1,000 days are critical period in human brain development and cognitive function acquisition potential, which is dependent on the available nutrition, health, social and cognitive stimulus. The development potential in children born and living in the slums, who are exposed to various adversities, can be mitigated through appropriate family-level practices with support from the community health workers and Nurses. This study is documenting the feasibility and impact of home visit linked coaching of families for improving child development status during the first 1,000 days in three sums of Delhi, India.

Neurodevelopmental disorders in children aged 2-9 years: Population-based burden estimates across five regions in India.

BACKGROUND: Neurodevelopmental disorders (NDDs) compromise the development and attainment of full social and economic potential at individual, family, community, and country levels. Paucity of data on NDDs slows down policy and programmatic action in most developing countries despite perceived high burden. METHODS AND FINDINGS: We assessed 3,964 children (with almost equal number of boys and girls distributed in 2-<6 and 6-9 year age categories) identified from five geographically diverse populations in India using cluster sampling technique (probability proportionate to population size). These were from the North-Central, i.e., Palwal (N = 998; all rural, 16.4% non-Hindu, 25.3% from scheduled caste/tribe [SC-ST] [these are considered underserved communities who are eligible for affirmative action]); North, i.e., Kangra (N = 997; 91.6% rural, 3.7% non-Hindu, 25.3% SC-ST); East, i.e., Dhenkanal (N = 981; 89.8% rural, 1.2% non-Hindu, 38.0% SC-ST); South, i.e., Hyderabad (N = 495; all urban, 25.7% non-Hindu, 27.3% SC-ST) and West, i.e., North Goa (N = 493; 68.0% rural, 11.4% non-Hindu, 18.5% SC-ST). All children were assessed for vision impairment (VI), epilepsy (Epi), neuromotor impairments including cerebral palsy (NMI-CP), hearing impairment (HI), speech and language disorders, autism spectrum disorders (ASDs), and intellectual disability (ID). Furthermore, 6-9-year-old children were also assessed for attention deficit hyperactivity disorder (ADHD) and learning disorders (LDs). We standardized sample characteristics as per Census of India 2011 to arrive at district level and all-sites-pooled estimates. Site-specific prevalence of any of seven NDDs in 2-<6 year olds ranged from 2.9% (95% CI 1.6-5.5) to 18.7% (95% CI 14.7-23.6), and for any of nine NDDs in the 6-9-year-old children, from 6.5% (95% CI 4.6-9.1) to 18.5% (95% CI 15.3-22.3). Two or more NDDs were present in 0.4% (95% CI 0.1-1.7) to 4.3% (95% CI 2.2-8.2) in the younger age category and 0.7% (95% CI 0.2-2.0) to 5.3% (95% CI 3.3-8.2) in the older age category. All-site-pooled estimates for NDDs were 9.2% (95% CI 7.5-11.2) and 13.6% (95% CI 11.3-16.2) in children of 2-<6 and 6-9 year age categories, respectively, without significant difference according to gender, rural/urban residence, or religion; almost one-fifth of these children had more than one NDD. The pooled estimates for prevalence increased by up to three percentage points when these were adjusted for national rates of stunting or low birth weight (LBW). HI, ID, speech and language disorders, Epi, and LDs were the common NDDs across sites. Upon risk modelling, noninstitutional delivery, history of perinatal asphyxia, neonatal illness, postnatal neurological/brain infections, stunting, LBW/prematurity, and older age category (6-9 year) were significantly associated with NDDs. The study sample was underrepresentative of stunting and LBW and had a 15.6% refusal. These factors could be contributing to underestimation of the true NDD burden in our population. CONCLUSIONS: The study identifies NDDs in children aged 2-9 years as a significant public health burden for India. HI was higher than and ASD prevalence comparable to the published global literature. Most risk factors of NDDs were modifiable and amenable to public health interventions.

Development assessment of HIV exposed children aged 6-18 months: a cohort study from North India.

HIV exposed children are vulnerable to developmental delay irrespective of their HIV status due to combined effect of risk factors like poverty, prenatal drug exposure, stress and chronic illness in family and malnutrition. This cohort study assessed the development of 50 HIV exposed children aged 6-18 months at a Pediatric Centre of Excellence in HIV care in India. The development was assessed using Development Assessment Scale for Indian Infants (DASII) at enrolment, 3 and 6 months later. The development quotient (DQ) scores and proportion of children with developmental delay (DQ ≤ 70) were compared among two sub-groups, HIV infected (HI) and HIV exposed uninfected (HEU) children. The various social and clinical factors affecting development were studied by univariate and multivariate analysis. Prevalence of developmental delay was 2.4% in the HEU (n = 41), and 33.3% in HI (n = 9). The DQ of HI was significantly lower than that of HEU at all three assessments. The DQ of HI were also significantly lower compared to the HEU at ages 12.1-18 months (83.37 ± 20.73 vs 94.68 ± 5.13, p = 0.005) and 18.1-24 months (84.55 ± 15.35 vs 94.63 ± 5.86, p = 0.006) respectively. The development of HEU was adversely affected by lower socioeconomic status and presence of wasting. In addition, development of HI was also adversely influenced by presence of stunting and opportunistic infections, advanced disease stage and shorter ART duration. We conclude that with optimum care, HEU can have a normal development, while a considerable proportion of HI may continue to have delayed development.

Validation of the Hindi Versions of Three Autism Specific Screening Tools (M-CHAT-R/F, RBSK-ASQ and TABC) Widely Used in India in 16-30-Month-Old Children.

OBJECTIVE: To determine the diagnostic accuracy of MCHAT-R/F, RBSK-ASQ and TABC for screening children aged 16 to 30 months for autism spectrum disorder (ASD). METHOD: Children aged 16 to 30 months were recruited from the pediatrics department. Those with known neurodevelopmental disorders, disabilities, severe medical illnesses, unavailable mothers, or lack of maternal understanding of Hindi, were excluded. The three index tools were translated into Hindi; each tool was piloted on 25 mothers and modified accordingly. The researcher was trained in administration, scoring and interpretation of the three tools. After enrollment the index tools and Developmental Profile (DP-3) were administered to each participant. The reference tool was a comprehensive assessment by experts that included clinical evaluation, computation of DP-3 scores, and application of diagnostic criteria of ASD; the final diagnosis being ASD or Non-ASD. RESULTS: Sensitivity and specificity of M-CHAT-R/F were 95.2% and 94.4%, of RBSK-ASQ were 100% and 93.9%, and of TABC were 100% and 94.4%, respectively. Convergent validity was high (Spearman's correlation coefficient 0.98). Test-retest and inter-rater reliability of each tool was excellent (Intra-class correlation coefficient 1.00). CONCLUSION: All three tools had acceptable psychometric properties, high convergent validity and excellent test-retest and inter-rater reliability.

Report of a novel Indian case of congenital erythropoietic porphyria and overview of therapeutic options.

Congenital erythropoietic porphyria is a rare disorder of heme biosynthesis, resulting from decreased enzymatic activity of uroporphyrinogen III synthase. Clinical manifestations are heterogenous, of variable severity, and with occasional phenotypic-genotypic correlation. A 14-month-old boy developed fever, extensive dermatitis, and reddish colored urine. Anemia, erythrodontia, hepatosplenomegaly, and massive urinary elimination of predominantly type I porphyrins was suggestive of congenital erythropoietic porphyria. Although hemolysis remained mild and compensated, facial and digital mutilation developed indicative of moderate clinical phenotype. Mutational analysis revealed compound heterozygosity of mutant alleles, including a novel mutation (p.Pro190Leu). The child received supportive management and underwent facial reconstruction successfully.

Diagnostic Accuracy of the Government of India Mother and Child Protection Card for Developmental Screening of Indian Children Aged 2-36 Months: A Hospital-based Mixed Method Study.

OBJECTIVE: Universal developmental screening is recommen-ded at 9, 18, 24 and 36 months. The Government of India Mother and Child Protection (MCP) card is an immunization record that is used to monitor child development, and identify children requiring further evaluation. OBJECTIVES: To determine the diagnostic accuracy of the MCP card for developmental screening, and perform its item analysis. STUDY DESIGN: Mixed-method study (prospective study of diag-nostic accuracy and qualitative study). PARTICIPANTS: Mother-child dyads of children between 2-36 months of age were recruited from the outpatient department or wards of a tertiary level children's hospital from November, 2019 to October, 2021. Children with confirmed neurodevelopmental disorders/disability, and mothers with less than 6th standard education were excluded. INTERVENTION: Each mother was given a MCP card, and taught how to mark the items. This was followed by the researcher's evaluation (index tool). The reference tool was a comprehensive clinical assessment (CCA) by the researcher and an expert. The CCA included clinical examination of hearing, vision, and neuro-development; and psychometric assessment of development and adaptive function. Each mother underwent an in-depth inter-view. Overall and group wise psychometric properties of diagnostic accuracy were computed. The interview transcripts were analyzed thematically. OUTCOMES: The proportion of children with 'fail' and 'delay' by the evaluation of the researcher with the MCP card and the expert by the CCA, respectively. RESULTS: The study population included 213 children (40.4% females). Fifty-two (24.4%) children were identified as 'Fail' by the MCP card and 43 (20.2%) as 'delay' by the expert's CCA. The overall sensitivity and specificity was 83.7% (95% CI 69.3-93.2) and 90.6% (95% CI 85.2-94.5), respectively. Acceptable diagnostic accuracy was found in the age-group 7-9 months, 13-18 months, and 25-36 months. CONCLUSIONS: The MCP card may be used for developmental screening at 9, 18, and 36 months.

Clinico-Etiologic Profile of Children and Adolescents with Drug-Resistant Epilepsy in a low-Resource Setting: 10 Years' Experience.

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.

Spectrum of Movement Disorders and Correlation with Functional Status in Children with Cerebral Palsy.

OBJECTIVES: To detail the spectrum of movement disorders (MD) among children with cerebral palsy (CP) and assess impact on functional status. METHODS: In this cross-sectional study, children with CP were recruited and examined for various MDs. Tone abnormality was assessed using Hypertonia Assessment Tool (HAT), functional status using Gross Motor Function Classification System Expanded and Revised (GMFCS E&R), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS). These scores were classified into mild-moderate (level I-III)/severe (level IV-V) categories. RESULTS: A total of 113 children (mean age 4.9 ± 3.4 y, 66.4% boys) were enrolled. MDs were noted in 52 (46%) children; the most frequent were dystonia (28%), chorea (14%), choreoathetosis (8%). Of 64 children with quadriparetic CP, 27 (42.2%) demonstrated MDs. Of 19 children with hemiparetic CP, 2 (10.5%) had MDs. Of 16 children with dyskinetic CP, 15 (93%) had MDs. Children with dyskinetic CP had significantly higher frequency of MDs (p = 0.001). There was no difference in occurrence of all MDs or dystonia aloneamongst the two categories (mild-moderate/severe) of GMFCS E&R levels, CFCS levels or MACS levels. CONCLUSION: Although diverse MDs occur frequently in CP, these do not correlate with the broad functional status of the child. The study is limited by small sample size.

Modified Atkins diet versus levetiracetam for non-surgical drug-resistant epilepsy in children: A randomized open-label study.

BACKGROUND: This study was undertaken to compare the efficacy of modified Atkins diet (MAD) among children with non-surgical drug-resistant epilepsy (DRE) to levetiracetam, when added to on-going anti-seizure medications. METHODS: An open-label, randomized controlled trial among children aged 2-12 years with non-surgical DRE was conducted. Eligible children were randomized in a 1:1 ratio to receive add-on MAD or levetiracetam. Baseline and post-intervention seizure frequency at 12 weeks was determined from seizure logs maintained by parents. The primary outcome was the proportion of responders, i.e., patients who achieved > 50% seizure reduction from baseline. Adverse events were compared. Analysis was intention-to-treat. (NCT04172311) RESULTS: One hundred and one children were enrolled (MAD-51, levetiracetam-50). The majority of the enrolled children had generalized seizures of mixed types secondary to structural brain lesions and Lennox-Gastaut syndrome was the most common electroclinical syndrome (46%). The proportion of children with >50% seizure reduction at 12 weeks was significantly higher in the MAD arm compared to the levetiracetam arm (27/51(52.9%) vs 11/50(22%); p < 0.001). At 12-weeks post-intervention, the change in mean seizure frequency compared to baseline was -47.33 ± 39.57% in the MAD arm and -31.15 ± 32.18% in the levetiracetam arm (p = 0.03). Constipation (41.1%) was the most frequent adverse effect with MAD. Sedation/lethargy (18%) and anxiety and irritability (14%) were the most frequent adverse effects in the levetiracetam group. CONCLUSION: Addition of MAD was found to be superior to levetiracetam among children with non-surgical DRE with predominant generalized seizures in achieving seizure reduction at 12 weeks. Both treatments were well tolerated. Adverse effects, although higher with MAD, were expected side effects.

Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study.

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.

Identification of Essential, Equivocal and Complex Autism by the Autism Dysmorphology Measure: An Observational Study.

The Autism Dysmorphology Measure is designed for non-expert clinicians. It uses an algorithm to assess 12 body regions and categorizes Autism on the number of dysmorphic regions identified; Essential (≤ 3), Equivocal (4-5) or Complex (≥ 6). We evaluated 200 Indian children with Autism (mean age 3.7 years) in a hospital-based cross-sectional study and compared inter-group profiles. We found 31% Essential, 49% Equivocal and 20% Complex Autism. On comparing results with existing literature, it appeared that genetic ancestry and age significantly influenced dysmorphism and hence categorization. No significant differences were observed between complex and essential autism in epilepsy, severity of autism or development, as reported earlier. These shortcomings make the present tool unsuitable for use in young Indian children with Autism.

Providing Services for Indian Children With Developmental Delay and Disabilities in the Community: Rashtriya Bal Suraksha Karyakram.

Investment in Early Childhood Development (ECD) is essential for the progress of a nation. In 2013, the Rashtriya Bal Suraksha Karyakram (RBSK) was launched for community level screening, early identification and management of chronic diseases (birth defects, diseases, deficiencies, developmental delays and disabilities) from birth to 18 years. Health care is provided in District hospitals with Special Newborn Care Units, and District Early Intervention Centers (DEIC). Infants are screened at delivery points, or at home under the Home-Based New-born Care package. Pre-schoolers and school aged children are evaluated by mobile health teams using standardized tools in anganwadi centers and schools, respectively. Referrals are managed at higher centers. The DEIC uses an evidence based, trans-disciplinary, collaborative approach for delay/disability at zero expense. Other initiatives disseminating awareness about healthy family practices promoting ECD during pregnancy and the first two years of life include: a booklet 'Journey of First 1000 days'; an android App 'Ayushman Bhava'; ECD call centers that provide individualized counselling related to queries; the LaQshya program that promotes mother-friendly labour; and a more illustrative 'Mother and Child Protection Card' that assists in developmental monitoring. Till date, RBSK has developed two Nodal Collaborating Centers (the Kolkata centre has trained 852 personnel), 234 DEIC's, and 11,000 mobile health teams. Over 6 years (2014 -2020), cumulatively 45,64,31,984 children < 6 years have been screened, 13,95,618 delays /disabilities identified, and 3,04,300 children managed appropriately. The future holds further expansion, development of state-of-the-art specialized centers, collaborative research, and self-sustaining capacity building of multi-disciplinary personnel.

Development and Evaluation of a Working Memory Intervention Kit in Children with Epilepsy in Low-Resource Settings.

OBJECTIVES: In this pilot study, the authors developed and evaluated a working memory intervention (WMI) using a combination of mobile phone-based application and an activity booklet, among children with idiopathic generalized epilepsy. METHODS: Pre- and post-intervention cognitive evaluation at 8 wk included: subtests comprising working memory index from Wechsler Intelligence Scale-IV, color cancellation task for sustained attention, and parent's rating from the Conners' ADHD/DSM-IV Scales of the Conners' Rating Scales-Revised. RESULTS: Fourteen children completed the intervention; one was lost to follow-up. Significant improvement in most working memory parameters occurred at 8 wk: digit span [scaled scores: median 7 (IQR 4-9) to 12 (IQR 9-14.25); p = 0.001]; letter-number sequencing [scaled scores: median 9 (IQR 5-10) to 11.5 (IQR 6.75-13); p = 0.03]; WMI [median 14 (IQR 9-18) to 22 (IQR 16.75-27); p = 0.001] and sustained attention [time for cancellation test improved from 95 (72-117) to 85 (63-98) s; p = 0.001]. CONCLUSION: This indigenous WMI was feasible and efficacious in improving working memory deficits in CWE in low-resource settings.

Evaluation of the Modified Atkins Diet for the Treatment of Epileptic Spasms Refractory to Hormonal Therapy: A Randomized Controlled Trial.

OBJECTIVES: We aimed to evaluate the efficacy of the modified Atkins diet in children with epileptic spasms who had failed hormonal therapy. METHODS: Children aged 9 months to 3 years having daily epileptic spasms despite a trial of ACTH or oral prednisolone and 1 additional anticonvulsant medication were enrolled. Children were randomly assigned to receive the modified Atkins diet either immediately or after a delay of 4 weeks. The ongoing anticonvulsant medications were continued unchanged. The primary outcome variable was the proportion of children who achieved spasm freedom as per parental reports at 4 weeks. Secondary outcomes included time to spasm cessation, proportion of children with electroclinical remission, the proportion of children with >50% reduction of spasms at 4 weeks, and adverse effects of the diet. (ClinicalTrials.gov Identifier: NCT03807141). RESULTS: A total of 91 children were enrolled in the study; 46 in the diet group and 45 in the control group. At the end of 4 weeks, 11 children in the diet group were spasm free compared with none in the control group (P ≤ .001). The median time to achieve spasm cessation was 10 days (interquartile range 9-20). Nine of these had resolution of hypsarrhythmia on electroencephalography (EEG). Thirty (65.2%) in the diet group had >50% reduction in spasms, compared with none in the control group (P < .001). The most common side effect was constipation, noted in 34.8% of the children. CONCLUSIONS: The modified Atkins diet was found to be effective and well tolerated in children with epileptic spasms refractory to hormonal therapy.

Intravenous Methylprednisolone Versus Oral Prednisolone for West Syndrome: A Randomized Open-Label Trial.

OBJECTIVE: To compare intravenous methylprednisolone (IVMP) with oral prednisolone (OP) for the treatment of West syndrome. METHODS: In this randomized, open-label trial, children aged 2 to 30 mo presenting with epileptic spasms with hypsarrhythmia or its variants on EEG were randomized to receive either IVMP (30 mg/kg/d for 3 d followed by oral prednisolone taper) or OP (4 mg/kg/d for two weeks followed by taper). The primary outcome measure was spasms cessation on day 14. Secondary outcomes included time to response, electroclinical remission at 2 and 6 wk, and frequency of adverse effects. ( ClinicalTrials.gov Identifier: NCT03876444). RESULTS: Sixty children were enrolled; 31 in the IVMP and 29 in the OP arm. Proportion of children achieving spasms cessation at day 14 was similar in both groups (54.8% versus 68.9%, p = 0.26). Time to achieve remission was lower in the IVMP group (mean 5.4 ± 0.9 versus 9.5 ± 2.6 d, p < 0.0001). Electroclinical remission at 2 wk was similar in both groups (51.6% versus 44.8%, p = 0.59) but lower at 6 wk in the IVMP group (45.2% versus 75.9%, p < 0.015). Adverse effects like sleep disturbance, irritability and hypertension were more common in IVMP group whereas weight gain was more common in the OP group. CONCLUSIONS: There was no significant difference in spasms cessation between the groups on day 14 although remission was higher at 6 wk in OP group. Our study suggests that OP was better than IVMP in efficacy and sustained remission with fewer adverse effects.

Electroclinical spectrum of childhood epilepsy secondary to neonatal hypoglycemic brain injury in a low resource setting: A 10-year experience.

PURPOSE: Neonatal hypoglycemic brain injury (NHBI) is being increasingly recognized as an important cause of drug resistant childhood epilepsy in low resource settings. We report the electro-clinical spectrum of children with epilepsy secondary to NHBI. METHODS: This was a retrospective study of children enrolled in the Epilepsy Clinic from January 2009 to August 2019. Data of children who had developed epilepsy after documented symptomatic neonatal hypoglycemia was collected. Details of clinical profile, seizure types, neurodevelopmental co-morbidities, EEG, neuroimaging findings and seizure outcomes were noted. RESULTS: One hundred and seventy children were enrolled. The mean age at seizure onset was 10.3 months (SD 0.5 months). The seizures types were epileptic spasms (76.5%), focal with visual auras (11.2%), bilateral tonic clonic (7.1%), myoclonic (3.5%) and atonic seizures (1.8%). The EEG findings included classical hypsarrhythmia (49.4%), hypsarrhythmia variant (27.1%), focal occipital or temporo-occipital spike wave discharges (10.6%), multifocal discharges (4.7%), diffuse slow spike and wave with bursts of fast rhythms (2.4%), continuous spike waves during sleep (1.2%) and normal EEG (4.7%). MRI showed gliosis with or without encephalomalacia in the occipital lobe with or without parietal lobe in 96.5% of the patients. Co-morbidities included global developmental delay (91.2%), cerebral palsy (48.7%), vision impairment (48.2%), microcephaly (38.2%), hearing impairment (19.4%), and behavioural problems (16.5%). Drug resistant childhood epilepsy was seen in 116 (68.2%) patients. CONCLUSIONS: Our study highlights the varied electroclinical and radiological spectrum and the adverse epilepsy and neurodevelopmental outcomes associated with NHBI.

Neuro-Developmental and Epilepsy Outcomes of Children with West Syndrome: A Cross-Sectional Study from North India.

OBJECTIVES: To assess the neurodevelopmental outcome of West syndrome (WS) in Indian children, who differ in their clinical profile from the western population. MATERIALS AND METHODS: This cross-sectional study enrolled children aged 2--5 years with prior diagnosis of WS between November 2013 and March 2015. They were assessed for epilepsy outcome and developmental outcome using developmental profile 3 (DP3) and vineland adaptive behavioral scale II (VABS II). RESULTS: Sixty-one children were enrolled. Perinatal asphyxia (40.9%), neonatal hypoglycemia (14.8%), and neonatal meningitis (9.8%) were predominant causes among the children with known etiology. Favorable epilepsy outcome (seizure freedom for >6 months) was observed in 29/61 patients (47.5%). Moderate to severe developmental delay was observed in 55/61 children (91.8%). Favorable developmental outcome (GDS by DP3 >70) was observed in just 5/61 (8%) patients. CONCLUSIONS: This study highlights the high prevalence of developmental delay in this population of children with WS, with adverse perinatal events being the most common etiology.

Use of the International League Against Epilepsy (ILAE) 1989, 2010, and 2017 Classification of Epilepsy in children in a low-resource setting: A hospital-based cross-sectional study.

OBJECTIVES: This cross-sectional study was designed to test the applicability of the 1989, 2010, and 2017 International League Against Epilepsy (ILAE) classification of epilepsy in children from a resource-limited setting in India. METHODS: Classification of seizure types and syndromes was done through parental interviews and review of medical records in children with epilepsy aged one month to 18 years. Available investigations including EEG, MRI, and metabolic/genetic tests were used in classifying patients as per the 1989, 2010, and 2017 ILAE (level II-epilepsy type) classification. We compared the proportion of children remaining unclassified by each scheme. RESULTS: Seven hundred and twenty-six children (436 males, mean age 6.4 ± 4.6 years) were enrolled. Using the 1989 ILAE classification, we were able to classify 95.7%, and 82.6% children by the 2010 scheme. The 2017 ILAE classification could classify all 726 children at level I (seizure type), 664 (91.0%) children at level II (epilepsy type), and an electroclinical syndrome could be identified in 409 (56.1%) of the children. An etiology could be identified in 75%, perinatal brain injury being the most frequent. West syndrome was the most common electroclinical syndrome, identified in 22.7% patients. The 1989 ILAE classification system was superior to the 2010 system (P = .01) in epilepsy classification. There was no difference between the 1989 and 2017 schemes (P = .31) or the 2010 and 2017 schemes (P = .10). SIGNIFICANCE: The 2017 ILAE classification, being multidimensional, allowed classification of children who could not undergo extensive evaluation due to economic constraints and also provided room for overlapping etiologies.

Rosai-Dorfman disease: a case with lymphadenopathy and liver involvement.

Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Formerly thought to be a disease process limited to lymph nodes, RDD has now been reported in many organ systems like bone, skin and soft tissue, central nervous system, eye and orbit, and upper respiratory tract. Here we report a case of RDD with hepatic involvement, which is even more rare.