title: [Clinical aspects and course of Sturge-Weber syndrome in childhood].
abstract: We report on symptoms, clinical course and prognostic factors of eight children (6 girls and 2 boys) with Sturge-Weber syndrome. The period of observation was 1 to 17 years (mean 8.8 years). All patients had the classical symptoms with facial port-wine stain nevus and intracranial calcifications. In two cases a bilateral distribution of the nevus was found. All patients but one had epileptic seizures which were followed by Todd's palsies in five. Four children additionally suffered from hydrophthalmia. Another patient had an angiomatosis of the retina and cataract. Neurological findings were normal in three patients, minimal signs were found in three, one patient had a spastic hemiplegia and another one hypotone tetraplegia. The mental development was normal in three patients, however severe dyscalculia occurred in one of them. Five patients were mentally retarded, three of them severely. The prognosis was the worse the earlier the cerebral seizures appeared.
mesh_terms: D000293:Adolescent; D000798:Angiomatosis / Q000175:diagnosis*; D001927:Brain Diseases / Q000175:diagnosis; D002114:Calcinosis / Q000175:diagnosis; D002648:Child; D002657:Child Development; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis; D007360:Intelligence; D008297:Male; D019965:Neurocognitive Disorders / Q000175:diagnosis; D009483:Neuropsychological Tests; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed
vernacular_title: Klinik und Verlauf des Sturge-Weber Syndroms im Kindesalter.