Abstracts

Sturge-Weber Syndrome (SWS) is a neurocutaneous disorder associated with a high incidence of seizures and intellectual problems. SWS is thought to be associated with a decline in cognitive funcions and research has indicated that the presence of seizures in individuals with SWS is associated with lower levels of intellectual functioning. This study attempted to separate the cognitive effects of SWS from those of a seizure disorder by evaluating the relative impacts of duration of SWS and duration of the seizure disorder on cognitive functioning. The isolated impact of SWS on cognitive functioning was also examined in a group of children with SWS who have never had a seizure., Data were collected as part of a survey study. Mothers and teachers of 66 chidren and adolescents with SWS and a seizure disorder were asked to complete questionnaires concerning cognitive and academic functioning. The results of intellectual assessments were available for 32 of these children. Mothers also provided information about medical and demographic characteristics. In addition, scores on the behavioral scales for a group of 14 children with SWS without a history of seizures were compared to those of a group of demographically matched normal controls., The duration of SWS was identiical to the age of the child at the time of intellectual assessment or when the questionnaire was completed but the age of the child was not significantly correlated with the duration of the seizure disorder. Duration of SWS was not significantly correlated with assessed IQ. Neither were parent or teacher reports of cognitive and academic problems correlated with duration of SWS. Duration of the seizure disorder, however, was inversely correlated with assessed IQ (p[lt].01) and positvely correlated with both parent and teacher reports of cognitive and academic problems (p[lt].01). Multiple regression analysis revealed that age of seizure onset explained an additional 11% of the variance in IQ scores. Duration of seizure disorder, age of seizure onset and seizure frequency explained 52% of the variance in parent report of intellectual problems. A comparison of children with SWS but no seizure disorder to normal controls did not reveal any significant differences in either parent or teacher report of cognitive and academic problems., Duration of seizure disorder rather than duration of SWS is associated with declines in intellectual functioning. Earlier age of seizure onset and seizure frequency are also associated with poor cognitive development. Children with SWS without a seizure disorder do not appear to be at risk for significant cognitive problems., (Supported by Sturge-Weber Foundation.)