STURGE-WEBER SYNDROME - AGE-OF-ONSET OF SEIZURES AND GLAUCOMA AND THEPROGNOSIS FOR AFFECTED CHILDREN

Data were obtained on 171 individuals with Sturge-Weber syndrome via q uestionnaire and medical records. The age of the study group ranged fr om 2 months to 59 years; the median was 8 years. In addition to the fa cial location of port-wine stains in the areas of the trigeminal derma tomes present in 170 patients, 45% also had extracranial port-wine sta ins over the torso and/or extremities, and 17% had other vascular or p igmentary lesions. Seizures were present in 80% of all patients (87% o f those with bilateral and 71% of those with unilateral port-wine stai ns); in all but one case, seizures were associated with port-wine stai ns in V-1 alone or V-1 and V-2 trigeminal dermatomes location. The age of onset of seizures ranged from birth to 23 years; 75% had onset of seizures before 1 year of age; these children had an 83% incidence of developmental and academic problems. Fifty-eight percent showed early developmental delay and required special education classes. The rate o f retardation showed a decreasing tendency with increasing age of onse t of seizures; of the children without seizures, only 6% had developme ntal delay and 11% required special education classes. Glaucoma was pr esent in 48% of patients (67% unilateral and 33% bilateral). Of all pa tients with glaucoma, 92% had port-wine stains in both V-1 and V-2 der matomes and 8% only in V-1. The laterality of glaucoma did not corresp ond to the trigeminal distribution of the port-wine stains in all inst ances. Glaucoma was diagnosed during the 1st year of life in 61% and b y 5 years in 72%; one patient did not have onset of symptoms until 38 years. The results of our data can serve as a guide for estimating rel ative risk figures for seizures, glaucoma, and mental retardation in c hildren with Sturge-Weber syndrome.