Scale of emotional development-short: Reliability and validity in two samples of children with an intellectual disability.

BACKGROUND: Intellectual disability (ID) is often accompanied by more significant delays in emotional development than in cognitive development. Diagnostic assessment can provide insight into emotional functioning. However, few standardized assessment instruments are available. AIMS: Examine the reliability and validity of the Scale of Emotional Development-Short (SED-S) in children with ID. METHODS AND PROCEDURES: This methodological instrument validation study was conducted in the Netherlands and Switzerland with children (N = 118) older than 3 and younger than 18 years with ID ranging from profound to mild. Measures included: demographic and medical data, SED-S, and the Vineland. Coherence and reliability of the SED-S were determined using Cronbach's alpha, and validity was examined using Goodman and Kruskal's γ, Kruskal-Wallis H, and Mann-Whitney U tests. OUTCOMES AND RESULTS: The reliability of the SED-S was high, the convergent validity was good, and divergent validity was indicated in relation to autism spectrum disorder (ASD), visual and/or auditory impairment, and adaptive functioning. FURTHER RESEARCH: Research is needed to better understand the implications of ASD and visual and/or auditory impairment on emotional development and their association with (normal) intelligence. Children with ID may also benefit from (more) detailed guidelines for imbalanced profiles on the SED-S.

Alexander disease--classification revisited and isolation of a neonatal form.

Alexander disease is usually classified according to the age of onset, e.g. an infantile form with onset during the first two years of life, a juvenile form with onset in childhood, mainly school age. It has been recognized, however, that the clinical course can be very variable within these groups. Thus, this clinical classification is not a useful predictor of severity and progression of the disease. This is demonstrated here on the basis of the history of seven own patients and a literature review. Only an onset in very early infancy, during the neonatal period, seemed to be associated with a rather uniform pattern of disease course, often leading to early death. This neonatal form showed very stereotyped symptoms, in part different from later onset: Early, often intractable, generalized seizures; hydrocephalus with raised intracranial pressure due to aqueductal stenosis because of pathological astroglia proliferation; lack of developmental progression but without prominent spasticity or ataxia; elevated CSF protein content. This was associated with the well-established neuroradiological findings, e.g. severe white matter affection with fronto-temporal predominance, involvement of basal ganglia and periventricular enhancement as an obligatory symptom. The identification of this early onset form is especially important as seizures and signs of raised intracranial pressure may mislead the diagnosis.