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 signifcantly 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.

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.

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. Neuroimaging, 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.

Screen Time in Under-five Children

Context:Screen-viewing in childhood is primarily a mean of entertainment, during the unstructured time. We aimed to review the burdenof the problem, delineate the associated factors and correlates, evaluate the impact of screen-time on the overall health of under-fivechildren, and the interventions to reduce screen-time. Evidence acquisition:Published articles from January 2009 to June 2018 weresearched through PubMed, Clinical Key, Scopus, Embase, and Google Scholar using key Medical Subject Heading words. Results: Theburden of screen-time varied from 21% to 98% in the middle-income, and 10% to 93.7% in the high-income countries. The socialecological model was used to illustrate associated factors and correlates including child, caregiver, micro and macro digital-mediaenvironment related factors. The interventions included increase in the physical activity, reduction in the body mass index, improvingsleep and dietary behaviors etc. The effectiveness of these interventions ranged from 0.3 minutes (standard error 13.3) to -47.16minutes (standard error 2.01). Conclusion: Clinicians should obtain history of screen-time in children, and advise limiting the screenexposure according to the child’s age. There is a need to generate evidence on burden and effectiveness of interventions amongchildren in the Indian settings, owing to the limited data.

Neurodevelopmental Outcome of Extremely Low Birth Weight Children at Corrected Age of Two Years.

Objective: To assess the neurodevelopmental, cognitive and behavioral function of extremely low birth weight babies (ELBW) till corrected age of two years. Methods: 79 ELBW babies were enrolled and followed at 1 year (n=50), 18 months (n=47) and 2 years (n=36). Adverse composite outcome was defined as death or moderate-to- severe neurodevelopmental impairment (defined as either cerebral palsy or DQ score <70 or deafness or blindness). Results: At 1 year, 24% were neurologically abnormal. At 18 months, average score (>85) was seen in 25 (54%) children in motor and 8 (17%) in mental development. Abnormal behavioral score (³12) was seen in 89% children. Adverse composite outcome was present in 28 (35.4 %) babies. Conclusion: ELBW neonates are at a high risk of neurodevelopmental and behavioral impairment.

Neurodevelopmental and behavioral outcome of very low birth weight babies at corrected age of 2 years.

Objective Neurodevelopmental and behavioral assessment of very low birth weight babies (VLBW) at corrected age (CA) of 2 years. Methods 127, 110, 99 and 101 babies ≤34 weeks and ≤1500 g were followed at CA of 3, 6, 9, 12 months respectively for developmental and neurological assessment. DASII (Developmental assessment scale for Indian infants) was used at CA of 18 months and preschool behavioural checklist (PBCL) at CA 2 years. Results Of 101 VLBW babies available for follow up at CA 1 year, 3 (3%) babies had Cerebral Palsy (CP) and 3% (n=3) had suspect abnormality (mild hypotonia), 11% (n= 11) had gross motor and 8% (n=8) had language abnormality. Their mean mental (MeDQ) and motor (MoDQ) quotients were 80.4±10.7 and 77.2±13.3 and a score of<70 was found in 17% (MeDQ) and 25.7% (MoDQ) VLBW babies. High PBCL score (mean 16.8± 5.4) was seen in 84%VLBW babies. On subgroup analysis, 2 babies (5%) in subgroup1 ( n=54, ≤1200 g,) and 1 (1.6%) in subgroup 2 (n=78, 1201–1500 g) had CP. Twelve (29%) in subgroup 1 had significant language delay (p=0.004) as compared to 4 (15%) in subgroup 2 at 1 year. BSID and PBCL scores were comparable. Amongst ELBW babies (<1000 g), 6.6% (n=1) had CP, 25% (n=3) and 42% (n=5) had low MeDQ and MoDQ respectively and all of them had high PBCL score. AGA and SGA had similar outcome. Conclusion VLBW babies need close and longer follow up due to high risk of neurodevelopmental and behavioral abnormality.

Multiple risks and early language development.

Objective. To examine the relationship between cumulative biological and environmental risk factors and the language development of children less than 3 years. Methods. A sample of 253 children aged 2 to 35 months, from an urban centre in north India were evaluated for language development. The main outcome measure was the language quotient (LQ) of the child as evaluated by the Clinical Linguistic Auditory Milestone Scale (CLAMS). Twelve possible risk factors, 4 biological and 8 environmental, were selected. Biological risk factors included preterm birth, low birth weight, history of birth asphyxia, and history of neonatal jaundice. The environmental risk factors included low income, large family size, minimal father’s and mother’s education, disadvantaged caste, low level of occupation of head of the household, absence of father, and higher birth order. A multiple risk score was created and categorized into 3 groups: low risk (0 to1), moderate risk (2 to 3) and high risk (4 and above). Results. There was a significant (F=4.80, p<.000) general downward linear trend in the LQ of the child as the number of risk factors increased. The difference in the LQ of the children with no risk factor and those with 8 risk factors was 21.21 LQ points and each risk factor reduced the LQ of the children by 2.63 points. Conclusion. Children experience many risk factors but the most detrimental effects on language development are caused when multiple biological and environmental risk factors act on a single child.

Developmental profile in children with iron deficiency anemia and its changesafter therapeutic iron supplementation.

Objective. To evaluate the developmental profile of children with iron deficiency anemia (IDA) and the changes following iron supplementation. Methods. Study was conducted prospectively in a tertiary care teaching institution. Subjects were children aged 6 months to 5-years, with IDA, proven by hematological parameters and iron studies. Complete blood counts and iron studies were performed at the beginning and following 3-months therapy with iron. Simultaneously, development was assessed by Developmental profile II (DPII), which was interpreted using IQ equivalent (IQE) scores and ‘fractional months differential’ (FMD). Results. Thirty five children fulfilled predetermined inclusion criteria. The mean-age was 22.3±13.4 months. Majority (71.4%) had moderate, while 5 (14.3%), each had mild and severe anemia. Significant developmental delay was observed in iron deficient children. Maximum delay was observed in academic and communication domains. 6 (17.2%) failed developmental screening, with IQE scores of <70. Significant improvement in DPII scores was noticed following therapy. Although some gain in IQE scores was noticed in the majority (88.6%), significant improvement (e =>10-point gain) was observed in about half (51.4%). Interpretation of DPII by FMD revealed significant improvement in all the domains as well. Conclusion. Children with IDA have suboptimal developmental scores. The delayed development is variably reversible following oral iron therapy. Hb =<7 g/dl and age >24 months predict suboptimal outcome. FMD is a useful method of interpreting DPII.

Impact of parent and teacher concordance on diagnosing attention deficit hyperactivity disorder and its sub-types.

OBJECTIVE: This study examines the extent to which parents and teachers agree on the diagnosis of Attention Deficit Hyperactivity Disorder (ADHD) and its subtypes, as per the DSM IV criteria. It assesses whether the extent of agreement between informants improves by making the ADHD criteria more flexible. METHODS: Parents and teachers of 119 clinic-referred sample of children (mean age=8.4 years, S.D= 2.48) with disruptive behavioral symptoms completed the Vanderbilt Attention Deficit Hyperactivity Disorder Diagnostic Parent and Teacher Rating Scales, respectively. Concordance of parent and teacher reports for the presence or absence of diagnosis of ADHD and type of ADHD was examined by percent agreement and the kappa statistics. RESULTS: Of the 119 children referred for disruptive behavior disorders, 96 (80.6%) met criteria for any type of ADHD according to the parents' report; and only 68 (57.1%) met criteria according to the teachers' report. Parent and teacher agreement for the diagnosis of any type of ADHD was only 52% (k= .11, n.s); and the agreement regarding diagnosis of sub-type was even poorer. Making the criteria more flexible vis-à-vis impairment or number of symptoms did not improve agreement between the informants. CONCLUSION: Clear guidelines are needed to reconcile the differences between informants in order to promote uniform diagnostic practices among clinicians working with children having ADHD.

Correlation of autism with temporal tubers in tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is an inherited genetic disorder commonly associated with neuropsychiatric complications like epilepsy, mental retardation, autism and other behavioral problems and constitutes about 1-4% of the autistic population. Mental retardation and seizures, particularly infantile spasms are significant risk factors for the development of autism. Patients of TSC with autism are more likely to have temporal tubers than those cases without autism. We describe clinical and neuroimaging features of two such cases of tuberous sclerosis with autism.

Multiple courses of antenatal steroids.

The benefits and risks of multiple courses of antenatal steroids (ANS) are still unresolved issues. This was a prospective cohort study in a level III neonatal unit. Preterm babies < or = 35 wk gestation were included. Malformations, chronic maternal steroid intake, exchange transfusions prior to cortisol sampling and incomplete ANS courses were exclusion criteria. Subjects were classified into: No course (Group 0), 1 course (group 1), 2 courses (Group 2), > 2 courses (Group 3) of antenatal dexamethasone. The key outcome was adrenal function assessed by basal and post-ACTH cortisol on day 3. Other outcomes were neonatal morbidity, mortality, growth parameters at birth, long term growth and neuro-development. Of 210 eligible babies, 124 were enrolled. 38, 51, 10 and 25 babies belonged to groups 0, 1, 2 and 3 respectively. Basal and post-ACTH serum cortisol did not show any significant difference between groups (p=0.5 and p=0.9 respectively). Incidence of severe HMD requiring ventilation was significantly lower (p=0.02) in multiple course group (combined groups 2 and 3) compared to single course group. There were no differences in other neonatal morbidity, birth OFC and weight between single and multiple ANS groups. Follow up data at a mean age of 22 mth was available in 59 subjects (69%) belonging to groups 1-3. No differences were noted in the proportion of patients with abnormal neurological examination (p=0.1), abnormal PDI (p=0.9), abnormal MDI (p=0.9) and physical growth between multiple and single course groups. Multiple courses of antenatal dexamethasone resulted in a significant decrease in severe forms of RDS and they did not cause adrenal suppression, decreased growth or impaired neuro-development.

Patterns and problems of sleep in school going children.

This study was conducted to assess the sleep habits and problems of 103 young school going healthy children (3-10 yr) during their visit to hospital for minor illnesses or routine health visits for immunization. The average duration of daily sleep (nocturnal and daytime nap) was 10.32 and the percentage of children who took regular daytime nap was 28.2%. Co- sleeping, a traditional cultural practice in India was found in 93% of the children. Sleep related problems were reported in 42.7% children that included nocturnal enuresis (18.4%), sleep talking (14.6%), bruxism (11.6%) nightmares (6.8%), night terrors (2.9%) snoring (5.8%) and sleepwalking (1.9%). On univariate analysis, sleep related problems were notably common if it was nuclear family (Fishers exact test; P = 0.01), mother was younger in age (Mann Whitney U test; P= .04) and less educated (Mann Whitney U test; P=.04). However, when these predictors were entered simultaneously into a logistic regression model, only nuclear family remained as significant predictor of sleep related disorders (odds ratio 2.41; CI; 1.04-5.57). We conclude that sleep problems are frequent among healthy school going children seen at general pediatric practice.

Maternal estimates of mental age in developmental assessment.

OBJECTIVE: To examine the accuracy and clinical utility of maternal estimates of mental age in young children referred for developmental assessment. METHODS: Mothers of 100 children aged 16 to 60 months referred for developmental evaluation to psychology services of Department of Pediatrics of a tertiary care teaching hospital were asked to estimate the mental age of their child. Maternal estimates were converted to intelligence quotient (IQ) and were compared to results from developmental tests of cognitive and adaptive behavior functioning which were administered to all children. RESULTS: Maternal estimate IQ was highly correlated with IQ calculated from Developmental Profile II (r=.83, p<.001) and social quotient (SQ) calculated from Vineland Social Maturity Scale (r=.81, p<.001). Maternal estimate IQ was 82% sensitive to cognitive delay and 81% specific in identifying children likely to have normal development. Twenty seven percent of the maternal estimates were within +/- 5 IQ points of actual IQ. Mothers were more likely to overestimate their child's functioning. Maternal IQ (Mean=62.1, S.D. =25.8) was significantly higher (t=2.93, p<.004) than the actual IQ (Mean=57.9, S.D.=21.9). Step-wise multiple regression analysis revealed that the child's IQ and SQ explained 10% of the variance (F=6.40, p<.001) in maternal accuracy. The lower the SQ and IQ of the child, more accurate the estimates. CONCLUSION: Maternal estimates of mental age provide an accurate measure of developmental functioning in young children and may be used as a screening technique to identify a subset of children who need more detailed evaluation.

Patterns of development in young children with autism.

OBJECTIVE: To determine the extent to which the developmental profile of children less than 4 years can help in distinguishing children with autism from children with developmental delay. METHODS: Subjects were 32 children with autism as per the DSM IV criteria and 32 children with developmental delay matched on chronological and academic age. The Developmental Profile II was used to assess the developmental functioning in five domains including physical, social, self help, academic, and communication. RESULTS: The two groups showed significantly different developmental profiles and these differences were accounted for mainly by significantly lower social skills and superior motor skills in the autistic group as compared to the developmentally delayed group. CONCLUSION: Developmental Profile II may help in distinguishing young children with autistic disorder from non-autistic children with comparable developmental delays.

Reactions of Indian adolescents to the 9/11 terrorist attacks.

OBJECTIVE: There is information about the impact of disasters and trauma on children, but little is known about the effects of terrorism particularly in India. (i) To assess the knowledge of the 9/11 terrorist attacks to the school going adolescents of India who were miles away from the actual incident. (ii) To compare the reactions to this event among the boys and girls. METHODS: The study used a survey design with a self-report questionnaire administered to 406 students in 6 schools of standards 9-12. The questionnaire was administered within 3 weeks of occurrence of this event. The mean age of the subjects was 16.34 years (SD= 1.22; range= 13-20) and 44.1% were boys. RESULTS: All the students were aware about this event. Awareness that the twin towers were hit was in 81.06 but only 51.94% knew that Pentagon was also hit. All the children knew who the prime suspect was although only 12.62% were aware about the country to which he belonged. The source of knowledge of the events was the television in 74.7% of the adolescents and 17.95% of them viewed foreign news channels additionally to the Indian channels to gather details about the event. Newspaper, radio and internet were the sources of information in 44.17%, 3.4% and 3.5% children respectively. Of the adolescents who gathered information from the television, 84.7% agreed that there had been an increase in their TV viewing time since the event and it was more than one hour per day in 47.5% of them. None of the students supported the terrorist attacks. The number of students with negative stressors was significantly more than the ones who were unaffected (p 0.0001). The girls were significantly more affected than the boys and while the former expressed anger the latter were more fearful and sad. (p 0.05) The idea of USA going for war against Afghanistan was supported by 69.4% and one third of them believed that such an event might adversely affect India. The adolescents who had witnessed the events on television were more fearful and shocked than the ones who read about the event in the print media (p 0.05). CONCLUSION: This study emphasizes the adverse reactions in the minds of adolescents in India to terrorist events even though they did not directly witness the events of September 11,2001.The role of media exposure in causing stress is also revealed. Pediatricians should be aware of the adverse effects of terrorism in the minds of the children and should be able to identify and help those who are in need.

Correlates of quality of life with epilepsy.

OBJECTIVE: To examine the quality of life of children with epilepsy and to identify the demographic, disease related, and behavioral and emotional functioning variables in the prediction of quality of life of children with epilepsy. METHOD: Forty three children aged 4 to 15 years (Mean=10.3 years) with epilepsy were recruited from the outpatient services of the Department of Pediatrics, of a tertiary care teaching hospital in North India. Quality of life was measured by Impact of Epilepsy Schedule, a 39 items parent reported questionnaire and child's emotional and behavioral functioning at home was assessed by the Childhood Psychopathology Measurement Schedule. RESULTS: Majority of the parents expressed major concerns regarding seizures, treatment by anticonvulsants, present and future problems for the child and problems in parenting. Nearly 40% of the children had psychopathology scores in the clinically significant maladjustment range. Step-wise multiple regression analysis revealed that the psychopathology scores and mother's education accounted for 39% of the variance in the quality of life scores. CONCLUSION: Children with epilepsy have a relatively compromised quality of life and focusing simply on control of seizures may not address the full range of child's emotional and behavioral difficulties.

Attention deficit hyperactivity disorder.

Attention deficit hyperactivity disorder (ADHD) is one of the most commonly diagnosed behavior disorder of childhood. In recent years, increasing number of preschoolers appear to be manifesting the core symptoms of ADHD. Diagnosis of ADHD in very young children is difficult as high activity level, impulsivity and short attention span are to some extent age appropriate characteristics of normal pre-school children. Concerns both about over-diagnosis and under-diagnosis have been expressed in the literature. Management emphasizing parental counseling, behavior management strategies, and appropriate pharmacotherapy is recommended.