GDD Global developmental delay OPD Outpatients departmentAIM To study the aetiology of intellectual disability in patients presenting to hospital and the diagnostic yield of a standardized examination.METHOD Over a 1-year period, the first three children presenting to the paediatric outpatients department (OPD) on 2 selected weekdays with developmental delay, suspected intellectual disability, or school failure were enrolled for study if they satisfied standard definitions of global developmental delay (GDD), or intellectual disability as tested by scales for Indian children: Developmental Assessment for Indian Infants, Binet Karnat Test, and the Vineland Social Maturity Scale (Malin's Adaptation). Detailed history, and physical and neurological examinations were recorded. An algorithmic approach to investigations was followed. Also, neuroimaging, thyroid function, electroencephalograph, karyotyping, and studies for fragile-X syndrome were conducted. Aetiological diagnosis was considered established only if clinical features were supported by investigations. Clinical features associated with a successful aetiological diagnosis were computed.RESULTS A total of 122 children were enrolled in a cross-sectional analytic study (mean age 43.5mo [SD 40.66]; 84 males, 38 females). Of these, a definite aetiology could be assigned in 66 children (54.1%); 17 prenatal, 38 perinatal ⁄ neonatal, and 11 postneonatal. Factors associated with reaching a definite diagnosis included younger age at presentation, presence of seizures, microcephaly, adverse neonatal events, and abnormal motor signs. Clinical history and examination gave important clues to the aetiology in 89 (72.9%) patients. Neuroimaging was abnormal in 91 out of 114 children, with aetiological findings in 48 children.INTERPRETATION Perinatal ⁄ neonatal causes predominate as the cause of GDD or intellectual disability in India. The study highlights that a large majority of cases seen here were preventable.Intellectual disability is characterized by significant limitations both in intellectual functioning and in adaptive behaviour as expressed in conceptual, social, and practical adaptive skills, which originate before age 18. 1 The term intellectual disability is used above the age of 5 years when standard psychometric testing becomes valid and reliable, while below this age the term global developmental delay (GDD) is used. 2 Intellectual disability is a disorder of varied aetiology broadly classifiable as having prenatal, perinatal, and postnatal causes. Aetiological yield of intellectual disability varies with its severity, the population and country studied, extent of diagnostic examination, and era in which the study was conducted. Overall, aetiological diagnosis is made in slightly over half the patients. 3,4 The reported frequencies of aetiological categories of intellectual disability are remarkably variable, with exogenous and endogenous (genetic) causes both ranging roughly between 17% and 47%. 5 Again, variations may be explained by differences in setting,...
GDD Global developmental delay LNDS Lucknow Neurodevelopment ScreenAIM To study prevalence and risk factors for neurological disorders -epilepsy, global developmental delay, and motor, vision, and hearing defects -in children aged 6 months to 2 years in northern India.METHOD A two-stage community survey for neurological disorders was conducted in rural and urban areas of Lucknow. After initial screening with a new instrument, the Lucknow Neurodevelopment Screen, screen positives and a random proportion of screen negatives were validated using predefined criteria. Prevalence was calculated by weighted estimates. Demographic, socio-economic, and medical risk factors were compared between validated children who were positive and negative for neurological disorders by univariate and logistic regression analysis. Neurological disorders are a leading cause of disability internationally. They have a considerable impact on the quality and duration of life. 1 Disorders of the brain may produce epilepsy as well as functional limitations in cognition, vision, hearing, and neuromotor ability. The same insult can cause disability in more than one domain and several different insults can affect one domain. Much of this disability is preventable at primary, secondary, or tertiary level. Prevalence of disability is dynamic and depends on factors such as age distribution of the population, lifespan of those affected, availability of preventive measures, and initial treatment received. RESULTS 2There are many causes of neurological disorders, which can be grouped as genetic, prenatal, perinatal, and postnatal. Many of the causes of neurological disorders are more common in resource-poor countries. Therefore, prevalence of neurological disability is expected to be higher in these countries but few studies have addressed this question in children. In resource-poor countries estimating the prevalence, epidemiology, and risk factors for neurological disorders is especially difficult owing to the paucity of health, mortality, and morbidity records. Such data, even if available, are often incomplete. In particular, data pertaining to infants below 2 years is largely unexplored so far. This age group is particularly challenging because neurological problems are difficult to pick up and are often evolving, which is why we prefer to use the term 'disorder' to 'disability' in this paper. However, because plasticity of a young child's brain allows a window of opportunity for remedial measures, it is important to study this age group.There is also a general lack of standardized screening tools for detecting neurological disorders in this age group. We have developed a tool -the Lucknow Neurodevelopmental Screen (LNDS) -to detect neurological disorders in this age group. A validation study of this tool in the hospital setting and then in the community has already been published. 3 The current paper reports the prevalence and risk factors for neurological disorders in this age group in the urban and rural community of Lucknow, India, using a t...
A chart was prepared using selected milestones from Baroda norms of Bayley Scales of Infant Development and Gesell's schedules on y-axis and age in months on x-axis. A child failing to achieve any milestone to the left of the chronological age was screen positive. Interrater and test-retest reliability were calculated. For validation, the screen was administered to mothers of 142 children aged 6 to 24 months attending Pediatric Outpatients or Neurology Clinic of CSM Medical University, Lucknow, India. Acutely ill children were excluded. A full Developmental Assessment Scale for Indian infants was then done on the same children. Sensitivity and specificity were 95.9% and 73.1%, respectively. It is concluded that Lucknow Development Screen can be effectively used for screening in the community.
Management of West syndrome is unsatisfactory. In our clinic we observed that a significant proportion of patients respond to usual dose of valproate. Objective: To prospectively assess the efficacy of valproate in controlling infantile spasms in West syndrome. Methods: Consecutive patients presenting with West syndrome to the Pediatric Neurology Clinic or general outpatient department (OPD) were enrolled for study. Those who were not on any treatment were given valproate in a dose of 30 mg/kg/day while awaiting investigations. Patients were followed up every 2 weeks. Predefined criteria for definition of West syndrome and response were used. Those showing partial/poor response or relapse on valproate were given hormonal therapy. Results: One hundred children with West syndrome were enrolled. Ninety one children were started on valproate. Of these 36 (39.5%) showed a good response, but seven later relapsed while on same dose of valproate and three were lost to follow up. Later age at onset and typical hypsarrhythmia on EEG were associated with good sustained response to valproate while a history of delayed cry at birth was associated with partial or poor response. Sixty two patients who responded poorly to or relapsed on valproate were put on hormonal treatment in addition. Of these 36 (58.1%) had a good response but 11 later relapsed after stopping treatment and two were lost to follow up. Conclusion: Valproate may have a role in treatment of West syndrome in a selected group of patients.
There is a dearth of comprehensive studies on comorbidities associated with intellectual disability (ID) in developing economy countries. This prospective study reports the prevalence of common comorbidities observed in a cohort of children with ID presenting to a regional medical center in Northern India. Subjects were drawn from the first three children between 6 months and 15 years of age presenting with complaints of developmental delay or mental dullness on one day a week at a pediatric outpatient unit and at the pediatric neurology clinic of a university teaching hospital. All potential subjects were tested for global developmental delay or ID by validated tests, and then the children were assessed for a set of predefined comorbid conditions, including epilepsy, cerebral palsy, microcephaly, impaired vision or hearing, feeding disorder, malnutrition, constipation, squint, drooling, violent behavior, autism, and attention deficit hyperactivity disorder (ADHD). The prevalence of comorbidities was found to be high, and it increased with severity of ID, except for ADHD, autism, and violent behavior, which decreased. Comparisons between children with and without cerebral palsy showed that most conditions were more common in children with cerebral palsy (in particular, epilepsy and constipation). A high prevalence of neurologic and medical comorbidities was seen in children with ID in Northern India, which underlines the need for a multidisciplinary approach to their identification and treatment. a Chi-square test b Significant. ADHD, attention deficit hyperactivity disorder; OR, Odds ratio; PEM, protein energy malnutrition.
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