Neurobehavioral characteristics of children with Duchenne muscular dystrophy

This study investigated cognitive, metacognitive, and psychosocial aspects of neurobehavioral functioning in 22 boys with Duchenne muscular dystrophy (DMD) and 18 unaffected siblings, all between the ages of 6 and 16 years. Probands and siblings completed the Wechsler Abbreviated Scale of Intelligence, as well as selected subtests from the Children's Memory Scale and from the Delis-Kaplan Executive Function System, while parents completed the Child Behavior Checklist and the Behavior Rating Inventory of Executive Function. Compared to siblings, probands demonstrated relative weaknesses on both verbal and nonverbal measures of delayed recall and response generation and were rated by parents as having more difficulties with social interaction, initiation, and adaptation. It is concluded that DMD is associated with mild but potentially significant difficulties in a range of neurobehavioral areas, likely related to deficient dystrophin levels in an integrated brain circuit that includes the cerebellum, hippocampus, and association neocortex.