NEUROBEHAVIORAL AND PSYCHOSOCIAL FUNCTIONING OF CHILDREN WITH OPSOCLONUS-MYOCLONUS SYNDROME

Childhood opsoclonus-myoclonus syndrome (OMS) is a movement disorder w hich typically strikes children in the early preschool years, seriousl y affecting intellectual, social-emotional and general adaptive develo pment. This series of 13 cases with well-documented neurological histo ries, aged 1.7 to 16.3 years, provides an initial systematic evaluatio n of these children's neuropsychological, psychosocial and adaptive st atus. As expected, children with OMS had significantly reduced intelli gence and severe speech and motor output problems; however, most of th em also demonstrated a range of preserved neurocognitive abilities and impressive goal-directedness and communicative effort. Psychosocial p roblems included mild behavioral impairment on the Achenbach Child Beh avior Checklist, and severe adaptive limitations on the Vineland Adapt ive Behavior Scales despite relatively strong social skills. Developme ntal factors and likelihood of subcortical localization are discussed, and practical guidelines are provided for behavioral and educational management of these children.