title: Cognitive and adaptive deficits in young children with Duchenne muscular dystrophy (DMD).
abstract: The goal of the current investigation was to examine adaptive behavior and cognitive skills in young children with Duchenne muscular dystrophy (DMD), a genetic disorder that causes progressive muscular weakness and concomitant cognitive deficits. Previous studies have documented specific language deficits in older children with DMD, but there are limited data on younger children. Twenty children with DMD who were between 3 and 6 years old and 20 unaffected family control children were recruited. Parents completed questionnaires relating to development and adaptive functioning, while children completed neuropsychological testing. Results of paired t tests indicate that children with DMD are rated as delayed relative to familial controls on measures of adaptive functioning, as assessed by the Vineland Adaptive Behavior Scales. Furthermore, children with DMD exhibit impairments on multiple measures of cognition, including measures of receptive language, expressive language, visuo-spatial skills, fine-motor skills, attention, and memory skills. Across all domains examined, the young children with DMD performed more poorly than their familial controls. These deficits appear to be more generalized than those reported in older children with this disorder. Dystrophin, a missing protein product, is hypothesized to be responsible for these cognitive and behavioral impairments.
authors.affiliation: The Graduate Center of the City University of New York, Manhattan, New York, USA. shanacyrulnik@pobox.com
authors.forename: Shana E
authors.initials: SE
authors.lastname: Cyrulnik
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Fee
authors.forename: Abigail
authors.initials: A
authors.lastname: Batchelder
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Kiefel
authors.forename: Edward
authors.initials: E
authors.lastname: Goldstein
authors.forename: Veronica J
authors.initials: VJ
authors.lastname: Hinton