Behavioral and academic problems in children with Sturge-Weber syndrome: Differences between children with and without seizures

Abstract

Although Sturge-Weber (SWS) syndrome is associated with behavioral and academic problems in childhood, it is unknown whether those problems are concomitants of the disorder itself or of the seizure disorder that is common in SWS. We compared two groups of children with SWS-- one with seizures (n = 20) and one without seizures (n = 14)--on parent-report and teacher-report measures of behavioral and academic functioning. The two subgroups were compared with each other as well as with children with epilepsy alone (n = 29) and a group of healthy controls (n = 21). The SWS group with seizures was more impaired than the seizure-free group on 9 of 15 measures and the children with seizures were 10 times as likely to have received special education services. Overall, children with SWS and seizures were similar to the epilepsy group, whereas children with SWS and no seizures were similar to the controls.

Introduction

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder identifiable by the presence of a distinctive triad consisting of vascular malformations of the eye, skin, and central nervous system.[1] Individuals with SWS are a highly heterogeneous group with respect to intellectual and behavioral functioning and there is only limited information about the factors that place particular individuals at risk for these associated problems. Those with lower levels of intellectual functioning are more likely to have a range of emotional and behavioral problems.[2], [3] The presence of a seizure disorder has been consistently associated with functional impairment although the findings for age of onset and seizure frequency have been inconsistent.[2], [4], [5], [6], [7], [8] The impact of seizures on cognition and behavior in individuals with SWS is not particularly surprising given the large body of research documenting the high incidence of cognitive and behavioral problems in individuals with epilepsy.[9], [10], [11], [12], [13], [14]

Given the link between seizure disorders and behavioral difficulties, and considering the high prevalence of seizure disorders in children with SWS, it is of interest to explore the developmental, academic and other behavioral functioning of children with SWS. Furthermore, little is known about whether young people with SWS who do not develop seizure disorders are at higher risk for cognitive and behavioral impairments than young people in the general population. The goal of the present study was to isolate the contribution of a seizure disorder, as distinct from the contribution made by SWS itself, to the reported pattern of developmental, academic, attentional and mood problems.

To this end we asked three questions: (1) Are problem behaviors more prevalent in young people with SWS who have seizures than those who have never had a seizure? (2) Are problem behaviors more prevalent in young people with SWS and a seizure disorder than other individuals with epilepsy who have a similar seizure burden and level of intellectual functioning? (3) Are young people with SWS who have never had a seizure at higher risk for behavior problems than individuals without a neurological disorder? To answer these questions, we used both parent and teacher measures of academic, social and behavioral functioning that would provide a broad-based assessment of behavioral development.