The Spectrum of Inherited Gray Matter Degenerative Brain Disorders (DBD) in Children: A Single-Center Study.

Objectives: To study the clinical spectrum of inherited gray matter degenerative brain disorders (DBD) in children. Methods: This cross-sectional study evaluated children up to 12 y of age, diagnosed with an inherited gray matter DBD in a tertiary care pediatric hospital between July 2019 and December 2020. Results: A total of 314 children with progressive neuroregression were screened. Of these, 117 children with inherited gray matter DBD were included in the study. The clinic-based prevalence of DBD was 8.2%, and inherited gray matter DBD was 3.1%. The proportion of the inherited gray matter DBD was 37.3% among the overall DBD cases. Children were categorized into three groups based on the age at onset of disease: below 2 years (N = 57, 48.7%), between 2 and 5 years (N = 32, 27.3%), and between 6 and 12 years (N = 28, 23.9%). Based on the predominant cerebral structure involved, gray matter DBD were classified as cerebral gray matter disorders (53%), basal ganglia disorders (34.1%), and cerebellar disorders (12.8%). Overall, the most common disorders were Wilson disease (18%), neuronal ceroid lipofuscinosis (NCL) (17%), and neurodegeneration with brain iron accumulation (NBIA) (16%). The most common gray matter DBD in children <2 years of age were NBIA (n = 11), Rett syndrome (n = 11), and gangliosidoses (n = 10). NCL (n = 14) and ataxia telangiectasia (n = 6) were most common in the age group of 2-5 years. Wilson disease (n = 19) was the most common disorder in the age group of 6-12 years followed by NCL (n = 4) and NBIA (n = 3). Conclusion: Our study highlights the burden and spectrum of gray matter DBD in children. The clinic-based prevalence of DBD was 8.2%, and of inherited gray matter DBD was 3.1%. The proportion of inherited gray matter DBD was 37.3% among the overall DBD cases. Wilson disease, NCL, and NBIA are the most common gray matter DBD in children. Timely diagnosis is important for the prevention of recurrence in subsequent pregnancies.

Cognitive Function in Early Onset Type 1 Diabetes in Children.

OBJECTIVES: To assess cognitive function and factors affecting it in Indian children with early-onset type 1 diabetes (T1D) (less than 6 y). METHODS: This cross-sectional, single-centre study recruited children diagnosed with T1D before 6 y of age and having a disease duration of at least 2 y, as cases. Controls were age- and sex-matched apparently healthy children or siblings. Children with birth asphyxia, intellectual disability, syndromic children, or pre-existing psychiatric illness were excluded. Enrolled children underwent cognitive assessment using Malin's Intelligence Scale for Indian Children (MISIC), and scores in various subtests were compared between cases and controls. RESULTS: A total of 60 children were enrolled in each group. When compared to controls, cases had significantly lower scores on most subtests, verbal, performance and overall Intelligence Quotient (IQ- 100.62 ± 3.26 vs. 103.23 ± 1.22). HbA1c >9%, severe hypoglycemia and lesser duration since the last diabetic ketoacidosis (DKA) episode significantly correlated with lower neurocognitive scores. CONCLUSIONS: Children with early onset T1D showed significant deficits in various cognitive domains and IQ. Poor glycemic control, higher glycemic variability and exposure to severe hypoglycemia are risk factors for poor cognitive outcomes in these children. Further longitudinal studies could potentially aid in a finer understanding of factors affecting cognitive functioning in T1D children in developing countries.

Exploring the genetic landscape of diphtheria, tetanus and pertussis vaccination-associated seizures or subsequent epilepsies.

Background: Diphtheria, Tetanus, and whole-cell Pertussis (DTwP) vaccination-associated seizures form the commonest type of serious adverse event following immunization in India and are an important reason for vaccine hesitancy. Our study explored the genetic explanation of DTwP vaccination-associated seizures or subsequent epilepsies. Methods: Between March 2017 and March 2019, we screened 67 children with DTwP vaccination-associated seizures or subsequent epilepsies, and of those, we studied 54 without prior seizures or neurodevelopmental deficits. Our study design was cross-sectional with a 1-year follow-up having both retrospective and prospective cases. We performed clinical exome sequencing focused on 157 epilepsy-associated genes and multiplex ligation-dependent probe amplification of the SCN1A gene at enrolment. We applied the Vineland Social Maturity Scale for neurodevelopmental assessment at follow-up. Findings: Of 54 children enrolled and underwent genetic testing (median age 37.5 months, interquartile range 7.7-67.2; diagnosis at enrolment: epilepsy 29, febrile seizure 21, and febrile seizure-plus 4), we found 33 pathogenic variants of 12 genes. Of 33 variants, 13 (39%) were novel. Most pathogenic variants were found in SCN1A gene (n = 21/33; 64%), SCN8A in 2 children, and 10 children had one variant in CDKL5, DEPDC5, GNAO1, KCNA2, KCNT1, KCNQ2, NPRL3, PCDH19, RHOBTB2, and SLC2A1. Five or more seizures (odds ratio [OR] = 5.3, confidence interval [CI]: 1.6-18.4, p = 0.006), drug-resistant epilepsy (OR = 9.8, 95% CI: 2.6-30.7, p = 0.001) and neurodevelopmental impairment (social quotient < 70) (OR = 5.6, 95% CI: 1.65-17.6, p = 0.006) were significant predictors of genetic diagnosis. Interpretation: Our study provides proof-of-concept for genetic aetiology in children with DTwP vaccination-associated seizures or subsequent epilepsies and has important implications for vaccination policies in developing countries. Funding: International Pediatric Association Foundation, Inc. (IPAF): Ihsan Dogramaci research award 2016/2017; Indian Council of Medical Research (ICMR), New Delhi, India: No.3/1/3/JRF-2016/HRD/LS/71/10940.

Neurodevelopmental Outcomes of a Cohort of Children with Tuberous Sclerosis Complex with Epileptic Spasms.

The neurodevelopmental outcomes in children with tuberous sclerosis complex (TSC) with epileptic spasms remain underdiagnosed and might be responsible for significant morbidity and mortality burdens, even after spasms abate. The study was a cross-sectional study over 18 months at a tertiary care pediatric hospital, involving 30 children with TSC who had epileptic spasms. They were assessed with Diagnostic and Statistical Manual of Mental Disorders-5 criteria for autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), and intellectual disability (ID), and childhood psychopathology measurement schedule (CPMS) for behavioral disorders. The median age at onset of epileptic spasms was 6.5 (1-12) months, and the age at enrolment was 5 (1-15) years. Of 30 children, 2 (6.7%) had only ADHD, 15 (50%) had only ID/GDD (global developmental delay), 4 (13.3%) had ASD and ID/GDD, 3 (10%) had ADHD and ID/GDD, and 6 (20%) had none. The median intelligence quotient/development quotient (IQ/DQ) score was 60.5 (20-105). CPMS assessment revealed significant behavioral abnormalities in almost half the children. Eight (26.7%) patients were completely seizure-free for at least 2 years, 8 (26.7%) had generalized tonic-clonic seizures, 11 (36.6%) had focal epilepsy, and 3 (10%) had evolved into Lennox-Gastaut syndrome. A high proportion of neurodevelopment disorders, including ASD, ADHD, ID/GDD, and behavioral disorders were seen in this pilot study with a small cohort of children with TSC with epileptic spasms.

Evaluation of a nurse-led counselling intervention on selected outcome variables for parents of children with congenital adrenal hyperplasia.

OBJECTIVES: Long-term care of children with congenital adrenal hyperplasia (CAH) has psycho-social implications for parents. Experts recommend a customized educational program for parents to facilitate their psychological adaptation and improve disease management. Such educational programs often provided by nurse counsellors are well evaluated in developed countries. There is a dearth of data on nurse-led counselling in the context of less developed countries. We aimed to evaluate the effect of a nurse-led counselling intervention on various psycho-social outcomes among parents of children with CAH. METHODS: Fifty consecutive parents of children with CAH attending an outpatient clinic at a tertiary-care teaching hospital were enrolled. Parents' stress level, stigma, knowledge, practices, and treatment adherence were assessed by using Cohen's Perceived Stress Scale (PSS), DSD Stigma scale, and HILL-Bon Medication Adherence Scale (HB-MAS). Three educational counselling sessions were conducted within a month's gap, using PowerPoint presentations and a booklet. Assessment of outcomes was done at baseline and at the end of the third session. RESULTS: At baseline, the majority (90%) of the parents had moderate stress. Half of the parents had mild and the rest had moderate stigma. After the intervention, the majority (94%) of parents had shifted to mild levels of stress and stigma. At baseline, 86% of the parents had poor knowledge about the disease whereas post-intervention, 80% were having good knowledge. Disease management practices and treatment adherence also improved after the intervention. CONCLUSIONS: Nurse-led counselling was effective in reducing psycho-social problems, increasing knowledge, as well as improving practices and treatment adherence.

Long-Term Neurological, Behavioral, Functional, Quality of Life, and School Performance Outcomes in Children With Guillain-Barré Syndrome Admitted to PICU.

BACKGROUND: Most children with Guillain-Barré syndrome (GBS) recover but may suffer from long-term sequelae, interfering with development and quality of life. Owing to the lack of published data, we aimed to assess the long-term neurological, behavioral, functional, quality of life, and school performance outcomes them. METHODS: Design: Cross-sectional observational. SETTING: Pediatric intensive care unit. PATIENTS: Children, aged one to 12 years, with GBS admitted over five years (July 2012 to June 2017) were enrolled during one year (July 2017 to June 2018). These children were assessed for the following outcomes: neurological (Hughes disability score, Pediatric Cerebral Performance Category [PCPC], Pediatric Overall Performance Category [POPC], and Glasgow Outcome Scale-Extended Pediatric version [GOS-E Peds] scales), behavioral (Childhood Psychopathology Measurement Schedule [CPMS]), functional (Vineland Social Maturity Scale [VSMS]-Indian Adaptation), quality of life (Pediatric Quality of Life [PedsQL]), and school performance (Parent-Directed Questionnaire). RESULTS: Eighty children were enrolled after a median of 3.0 (1.3-4.2) years from discharge. The majority (95%) had favorable neurological recovery (Hughes disability score 0 to 1). Favorable outcome was noted in 95% of children on PCPC, 87.5% on POPC, 60% on GOS-E Peds, 86.2% on CPMS, 92.5% on VSMS, and 98% on PedsQL. The majority (97.5%) of childre were attending schools, and 57.7% had satisfactory school performance. The presence of quadriparesis at admission, mechanical ventilation, tracheostomy requirement, poor ambulatory status at discharge, and longer pediatric intensive care unit and hospital stay predicted unfavorable neurological outcome on different tools. Absence of quadriparesis at admission and no requirement of mechanical ventilation predicted a favorable result on all outcome measures. CONCLUSIONS: On long-term follow-up, most children with severe GBS showed favorable neurological, behavioral, functional, and quality of life outcomes. Severe clinical presentation and prolonged intensive care unit stay predict poor long-term outcome.

Early Diagnosis of Autism Spectrum Disorder: What the Pediatricians Should Know.

Autism is a spectrum disorder marked by considerable heterogeneity and characterized by impairments in the social communication domain along with the presence of restricted and repetitive behaviors or interests. Comprehensive autism evaluation generally consists of assessments by a multidisciplinary team. Having multiple specialists in the evaluation team aids in diagnosis and in chalking out a comprehensive management plan. Diagnosis is generally based on detailed developmental history, clinical judgment, and the use of standardized diagnostic instruments. Differential diagnosis is complicated as many of the mental health and neurodevelopmental conditions that routinely coexist with autism also have some symptoms that overlap with autism. Several barriers are linked to delay in diagnosis including lack of comfort in diagnosing autism by primary care providers, delayed referrals, the inability of parents to raise critical developmental concerns, confusion of autism with other conditions, and health system that is not responsive to the needs of the underserved communities. The etiology of autism spectrum disorder (ASD) is complex and still not completely understood; it involves genetics, neurobiology, and environmental exposures, leading to a diverse presentation of behaviors and symptoms. There is an imperative need to start therapeutic interventions as soon as a diagnosis of autism is suspected rather than wait for a definitive diagnosis. Early diagnosis is vital as timely intervention can lead to better outcomes for children and their families.

A Randomized, Controlled, Noninferiority Trial Comparing Vitamin B12 Monotherapy Versus Combination Multinutrient Therapy with Vitamin B12 for Efficacy in Treatment of Infantile Tremor Syndrome.

OBJECTIVES: To compare the mean Likert (caregiver impression of change) and CAPUTE scores in children with ITS treated with daily injectable vitamin B12 alone versus injectable vitamin B12 with other multinutrients at 1 wk and 1 mo of therapy. METHODS: This was an open-label, active-controlled, assessor-blinded, noninferiority, randomized clinical trial. The participants included children aged 3 mo to 2 y with infantile tremor syndrome. Children were randomized to receive either 1 mg of daily injectable vitamin B12 or 1 mg of daily injectable vitamin B12 with other multinutrients (B12 + MV). Primary outcome measure was the mean Likert score in the two arms at 1 wk. Secondary outcome measures were mean change in CAPUTE scores at 1 wk of therapy; and mean change in CAPUTE and Vineland Social Maturity Scale (VSMS) scores after 1 mo of treatment. RESULTS: Seventy-two (N = 72) of the 160 screened were enrolled and randomized. The mean (SD) Likert score in the B12 group (n = 38) was 16.1 (3.7) and in the B12 + MV group (n = 34) was 14.9 (3.7); p = 0.237. Mean (SD) change in CAPUTE (CAT/CLAMS) at 1 mo in the groups was not statistically different. The mean (SD) change in social quotient in the B12 monotherapy group, 35.0 (20.7) was significantly higher than the B12 + multinutrient group 23.5 (15.4); p=0.01. CONCLUSION: Injectable vitamin B12 monotherapy in ITS resulted in an improvement that was noninferior to combination multinutrient therapy, strongly supporting vitamin B12 deficiency as the cause of infantile tremor syndrome. TRIAL REGISTRATION: The trial was registered at CTRI.org (CTRI/2018/05/013841).

Sleep Problems in Children With Neurocutaneous Syndromes: A Cross-Sectional Study.

Introduction: The prevalence and patterns of sleep disturbances in neurocutaneous syndromes are variable and understudied. Methods: Cross-sectional study for 18 months at a tertiary care pediatric hospital, involving 100 children with neurocutaneous syndromes aged between 4 and 10 years using the Children's Sleep Habits Questionnaire-Abbreviated. Results: In 100 children with neurocutaneous syndromes, 47 (47%) had significant sleep problems. In subgroup analysis, 7 of 17 children with neurofibromatosis 1, 24 of 63 children with tuberous sclerosis complex, 10 of 12 children with Sturge-Weber syndrome, 2 of 3 children with linear nevus sebaceous syndrome, and each of the children with hypomelanosis of Ito, McCune-Albright syndrome, megalencephaly-capillary malformation syndrome, and unclassified neurocutaneous syndrome had significant sleep problems. Conclusion: The prevalence of sleep problems in our study population was not more than that observed in the general pediatric population. Prospective multicentric studies are needed to comprehend sleep problems in children with neurocutaneous syndromes.

Cutaneous and ocular manifestations in Indian children with neurocutaneous syndromes: A cross-sectional study.

The phenotypical profile of cutaneous and ocular manifestations in neurocutaneous syndromes is inconstant. We made a cross-sectional study over 18 months of children with neurocutaneous syndromes aged between 1-15 years. A varied presentation was found. Attention to both the typical but also the rare atypical presentations enhances an early identification of the disorder and therefore opportune management.

Parenting a child with autism spectrum disorder: A qualitative study.

Background: Research in India has seldom studied caregivers' perceptions, experiences, and needs for information and personal support after an autism spectrum disorder (ASD) diagnosis. Objectives: The objectives of the study were to understand the perceived barriers for obtaining a diagnosis and the perspectives and experiences of parents of children with autism. Materials and Methods: Parents with a diagnosed ASD child (within a year of diagnosis) in the 3-8 years range were recruited from the Pediatric Psychology and Neurodevelopmental Clinic from a tertiary care teaching hospital in North India. An interview guide elicited information about experiences regarding obtaining an ASD diagnosis, perceived barriers and facilitators, reactions to diagnosis, postdiagnostic family and community experiences, and stress experienced by parents. Qualitative responses were analyzed using thematic analysis. Participants were recruited till there was a saturation of themes. The ethics clearance was provided by the institutional review board. Results: Twenty-eight caregivers of children with ASD were recruited for the study. Overall, nine themes were identified from the qualitative analysis of the interviews: two before diagnosis (delayed help-seeking and experiences with healthcare), one at the time of diagnosis disclosure (heightened emotional response to diagnosis), and six themes after the diagnosis (increased stress, behavioral challenges, deterioration in family relationships, negative attitudes of the family, seeking support, and moving forward with hope for the future). Conclusions: There are several barriers and gaps in the autism-related available services in the country, and there is a need to provide inclusive, supportive, culturally sensitive, and family-centered model of care for parents raising children with ASD.

Restricted and Repetitive Behaviors and Interests in Young Children with Autism: A Comparative Study.

OBJECTIVES: To examine the frequency of repetitive behaviors among children with autism spectrum disorder (ASD) and compare it to age-matched developmentally delayed (DD) and typically developing children (TD). METHODS: Twenty-five children (3-8 y) with a diagnosis of ASD were compared to two age-matched control groups namely the DD and TD groups. The Repetitive Behaviors Scale-Revised (RBS-R) was administered to the parents to assess the frequency, severity, and impairment associated with restricted, repetitive behaviors, and interests (RRBIs) displayed. The study was cleared by the institute ethics committee. RESULTS: The three groups were well matched on age and sex distribution and comparisons on the RBS-R showed that the ASD group had a significantly higher total score (F = 51.52, p = 0.0001) and number of items endorsed on the scale (F = 37.91, p = 0.0001). Stepwise multiple regression analysis revealed that 35.8% of the variance in the total RRBI score of the ASD children was explained by the age and severity of autism (F = 6.12. p = .008). Older children with severe ASD features had significantly higher total RRBI scores. CONCLUSIONS: RRBIs are not just specific to autism and should not be used in isolation to identify children with autism. Gaining a complete understanding of the complexity and variability of the restricted interests would help in improving the diagnostic procedures and in the development of a comprehensive therapeutic plan.

Identification of novel pathogenic variants in the GCDH gene and assessment of neurodevelopmental outcomes in 24 children with glutaric aciduria type 1.

AIM: To evaluate the pathogenic variants in GCDH gene and to assess the neurodevelopmental outcomes in children with Glutaric aciduria type 1 (GA-1). METHOD: Cross-sectional observational study between January 2019 and June 2020 in consecutive North Indian children with a clinical and biochemical suspicion of GA-1. Variants in the coding regions of GCDH gene were identified through Sanger sequencing. Neurodevelopmental and quality of life assessment was done using standardized scales. RESULTS: 24 children with GA-1 were identified. The median age at diagnosis was 12 months and the median delay in diagnosis was 3 months. Genetic analysis was done in 14 cases. It revealed 12 variants (11 missense and one nonsense) from 13 patients. Most of the pathogenic variants were in exon 9 and exon 5. Three novel variants were identified in three patients: two missense variants c.169G > A (p.Glu57Lys), c.1048T > C (p.Cys350Arg) and one nonsense variant c.331C > T (p.Lys111Ter). On neurodevelopmental assessment, majority of children with GA-1 were non ambulatory (62.5%), had limited hand skills (58.3%) and impaired communication (58.3%). Overall, poor global development was noted in 43.7%. A pre-existing developmental delay was significantly associated with impaired communication skills (p = 0.03), and the number of episodes of encephalopathy were significantly associated with impaired gross motor skill (p = 0.02). Presence of encephalopathy was significantly associated with poor performance in social emotional (p = 0.01) and cognitive (p = 0.03) domains of Developmental Profile-III scale and development of severe dystonia (p = 0.01). CONCLUSION: Our findings highlight the clinical, biochemical, radiological and genetic spectrum of GA-1 in children in North India and report the presence of novel pathogenic variations.

Diagnosis and Management of Global Development Delay: Consensus Guidelines of Growth, Development and Behavioral Pediatrics Chapter, Neurology Chapter and Neurodevelopment Pediatrics Chapter of the Indian Academy of Pediatrics.

JUSTIFICATION: Global developmental delay (GDD) is a relatively common neurodevelopmental disorder; however, paucity of published literature and absence of uniform guidelines increases the complexity of clinical management of this condition. Hence, there is a need of practical guidelines for the pediatrician on the diagnosis and management of GDD, summarizing the available evidence, and filling in the gaps in existing knowledge and practices. PROCESS: Seven subcommittees of subject experts comprising of writing and expert group from among members of Indian Academy of Pediatrics (IAP) and its chapters of Neurology, Neurodevelopment Pediatrics and Growth Development and Behavioral Pediatrics were constituted, who reviewed literature, developed key questions and prepared the first draft on guidelines after multiple rounds of discussion. The guidelines were then discussed by the whole group in an online meeting. The points of contention were discussed and a general consensus was arrived at, after which final guidelines were drafted by the writing group and approved by all contributors. The guidelines were then approved by the Executive Board of IAP. Guidelines: GDD is defined as significant delay (at least 2 standard deviations below the mean with standardized developmental tests) in at least two developmental domains in children under 5 years of age; however, children whose delay can be explained primarily by motor issues or severe uncorrected visual/hearing impairment are excluded. Severity of GDD can be classified as mild, moderate, severe and profound on adaptive functioning. For all children, in addition to routine surveillance, developmental screening using standardized tools should be done at 9-12 months,18-24 months, and at school entry; whereas, for high risk infants, it should be done 6-monthly till 24 months and yearly till 5 years of age; in addition to once at school entry. All children, especially those diagnosed with GDD, should be screened for ASD at 18-24 months, and if screen negative, again at 3 years of age. It is recommended that investigations should always follow a careful history and examination to plan targeted testing and, vision and hearing screening should be done in all cases prior to standardized tests of development. Neuro-imaging, preferably magnetic resonance imaging of the brain, should be obtained when specific clinical indicators are present. Biochemical and metabolic investigations should be targeted towards identifying treatable conditions and genetic tests are recommended in presence of clinical suspicion of a genetic syndrome and/or in the absence of a clear etiology. Multidisciplinary intervention should be initiated soon after the delay is recognized even before a formal diagnosis is made, and early intervention for high risk infants should start in the nursery with developmentally supportive care. Detailed structured counselling of family regarding the diagnosis, etiology, comorbidities, investigations, management, prognosis and follow-up is recommended. Regular targeted follow-up should be done, preferably in consultation with a team of experts led by a developmental pediatrician/ pediatric neurologist.

Evaluation of Hyperandrogenism in Children with Autism Spectrum Disorder.

This study evaluated 32 children (mean age: 8.5 y) with autism spectrum disorder (ASD) and 23 healthy controls (similar age, sex, and Tanner stage) for hyperandrogenism. These children underwent sexual maturity rating (Tanner staging), ASD severity assessment (Childhood Autism Rating Scale), and quantitative estimation for plasma testosterone, dehydroepiandrosterone sulfate (DHEAS), and androstenedione. There was no significant difference in androgen levels in the two groups. Elevated (> 95 centiles) testosterone, DHEAS, and androstenedione levels were seen in 12.3%, 6.2%, and 9% children with ASD, and 7/9 of these children (78%) with hyperandrogenism had severe ASD. However, there was no significant correlation between ASD severity and androgen levels.

Comparison of Long-Term Outcomes Between 7 Days and 28 Days Albendazole Monotherapy in the Treatment of Single-Lesion Neurocysticercosis in Children.

OBJECTIVES: The objective was to compare the long-term clinical, radiological, and cognitive outcomes in children with single-lesion neurocysticercosis who received 7 or 28 days of albendazole therapy. METHODOLOGY: This observational study conducted over 1 year included (1) consecutive children with single-lesion neurocysticercosis who received 7 or 28 days of albendazole therapy in the acute state and (2) completed follow-up for at least 5 years. Seizure recurrence, resolution of lesions, cognition (Malin's Intelligence Scale for Indian Children), behavior, and school performance (National Initiative for Children Healthcare Quality Vanderbilt Assessment Scale) were assessed. RESULTS: Group A (albendazole for 7 days) comprised 55 children, and group B (albendazole for 28 days) included 48 children. The mean age at the time of diagnosis of neurocysticercosis was 6.6 ± 1.8 years; the mean age at the time of assessment for the study was 13.2 ± 1.2 years. Focal-onset seizures were the most common clinical presentation (58.3%). The majority of lesions were ring-shaped (92.3%) or colloidal (58.2%), with perilesional edema (89.3%). In the long-term follow-up, radiological resolution of the lesions was comparable in both groups. Complete resolution was seen in 52.7% receiving 7 days and 54.2% receiving 28 days albendazole. Seizures recurred in 20% receiving 7 days and 20.8% receiving 28 days albendazole. Overall, a low intelligence quotient (IQ < 70) was seen in 55.3% cases, "somewhat problematic" school performance in 12%, and behavioral abnormalities were present in 20% of the cases. The results were comparable between the 2 groups. CONCLUSION: Seizure control, radiological resolution of lesion, school performance, cognitive and behavioral outcomes in the long term are comparable in children with single-lesion neurocysticercosis who have received albendazole cysticidal therapy for 7 days and 28 days. Recurrence of seizure is seen with both regimens in the long term, necessitating regular follow-up and discussion regarding the risk of recurrence before a withdrawal of anticonvulsant therapy.

Epilepsy and Neurodevelopmental Outcomes in a Cohort of West Syndrome Beyond Two Years of Age.

OBJECTIVE: To determine epilepsy and neurodevelopmental outcomes beyond 2 y of age and their putative prognostic factors in children with West syndrome (WS). METHODS: This cross-sectional study was initiated after approval from Institutional Ethics Committee. A follow-up cohort of 114 children (aged ≥ 2 y) diagnosed and treated for WS at the authors' center were assessed in-person for epilepsy and neurodevelopmental outcomes using Vineland Social Maturity Scale - Malin's adaptation for Indian children. Subsequently, age at onset, lead-time-to-treatment, etiology, and response to any of the standard therapies were analyzed as possible predictors of these outcomes. RESULTS: Of 114 children (mean age: 55 ± 32 mo, 91 boys), structural etiology was the predominant underlying etiology (79.8%) for WS. At 2 y of age, 64% had ongoing seizures. At the last follow-up, 76% had social quotient < 55, and 39% had cerebral palsy (spastic quadriparesis in 21%). An underlying structural etiology was associated with ongoing seizures [OR (95% CI) 3.5 (1.4-9); p = 0.008] at 2 y of age and poor developmental outcomes [OR (95% CI): 3.3 (1.3-8.9); p = 0.016]. Complete cessation of spasms with the standard therapy was significantly associated with better seizure control [OR (95% CI): 5.4 (2.3-13); p < 0.001] and neurodevelopmental outcome [OR (95% CI): 5.2 (1.8-14.9); p < 0.001]. CONCLUSION: The majority of children with WS have a poor neurodevelopmental outcome and epilepsy control on follow-up. The underlying etiology and response to initial standard therapy for epileptic spasms have a prognostic role in predicting the neurological outcome in these patients on follow-up.

Designing a multi-component 'Stop Bullying-School Intervention Program' in Chandigarh, a North Indian Union Territory.

Bullying, a prevalent global public health issue, is proven to have an adverse impact on the physical and psychological health of school students. There are few intervention programs to prevent bullying in the South East Asian Region, and none in India. The objective of this study was to design a multi-component antibullying intervention program known as 'Stop Bullying-School Intervention Program (SB-SIP)' for school students. It was developed in five stages. Stage one was the review of existing literature on intervention studies to prevent bullying, globally. A qualitative study to explore the beliefs and perceptions of teachers, students, and parents regarding antibullying intervention programs was conducted in stage two. In the third stage, a conceptual model was framed. A consultation workshop was conducted to finalize the contents of the intervention in the fourth stage. Pretesting of the intervention was done in the fifth stage. The literature review provided evidence that a whole-school intervention program based on the socio-ecological model was the most effective. The awareness of the effects of bullying and effective strategies to prevent it in the school setting was suggested to be part of the SB-SIP by the majority of the participants in the focus group discussions. The recommendations given by the stakeholders in the consultation workshop contributed mainly to the method of delivery of the program. The five-stage process helped in recognition of the conceptual model and modifiable factors, which exerts its effects on bullying and its psychosocial outcome, through which the multi-component antibullying intervention program SB-SIP was finalized.