Neurodevelopment of Children Under 3 Years of Age With Smith-Magenis Syndrome

Systematic data regarding early neurodevelopmental functioning in Smith-Magenis syndrome are limited. Eleven children with Smith-Magenis syndrome less than 3 years of age (mean, 19 months; range, 5-34 months) received prospective multidisciplinary assessments using standardized measures. The total s...

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Bibliographic Details
Published in:Pediatric neurology 2009-10, Vol.41 (4), p.250-258
Main Authors: Wolters, Pamela L., PhD, Gropman, Andrea L., MD, Martin, Staci C., PhD, Smith, Michaele R., PT, MEd, Hildenbrand, Hanna L., MS, Brewer, Carmen C., PhD, Smith, Ann C.M., MA, DSc (Hon)
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child Behavior
Child Development
Child Language
Child, Preschool
Cognition
Female
Genetic Diseases, Inborn - pathology
Humans
Infant
Male
Malformations of the nervous system
Medical sciences
Motor Activity
Neurologic Examination
Neurology
Neuropsychological Tests
Pediatrics
Syndrome
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Summary:Systematic data regarding early neurodevelopmental functioning in Smith-Magenis syndrome are limited. Eleven children with Smith-Magenis syndrome less than 3 years of age (mean, 19 months; range, 5-34 months) received prospective multidisciplinary assessments using standardized measures. The total sample scored in the moderately to severely delayed range in cognitive functioning, expressive language, and motor skills and exhibited generalized hypotonia, oral-motor abnormalities, and middle ear dysfunction. Socialization skills were average, and significantly higher than daily living, communication, and motor abilities, which were below average. Mean behavior ratings were in the nonautistic range. According to exploratory analyses, the toddler subgroup scored significantly lower than the infant subgroup in cognition, expressive language, and adaptive behavior, suggesting that the toddlers were more delayed than the infants relative to their respective peers. Infants aged approximately 1 year or younger exhibited cognitive, language, and motor skills that ranged from average to delayed, but with age-appropriate social skills and minimal maladaptive behaviors. At ages 2 to 3 years, the toddlers consistently exhibited cognitive, expressive language, adaptive behavior, and motor delays and mildly to moderately autistic behaviors. Combining age groups in studies may mask developmental and behavioral differences. Increased knowledge of these early neurodevelopmental characteristics should facilitate diagnosis and appropriate intervention.
ISSN:0887-8994
1873-5150
DOI:10.1016/j.pediatrneurol.2009.04.015