title: Clinical outcomes in bilateral Sturge-Weber syndrome.
abstract: Approximately 15% of patients with Sturge-Weber syndrome demonstrate bilateral intracranial involvement, and the prognosis of these patients is considered particularly unfavorable. We reviewed the clinical and neuroimaging features of patients with Sturge-Weber syndrome and bilateral intracranial involvement. Seizure variables, the presence of hemiparesis, and the degree of developmental impairment at most recent follow-up were compared with imaging abnormalities. Of 110 Sturge-Weber syndrome patients, 14 demonstrated bilateral brain involvement, with an asymmetric pattern on glucose metabolism positron emission tomography. Although most patients manifested frequent seizures initially, associated with frontal hypometabolism on positron emission tomography, six (43%) had achieved good seizure control during follow-up. Bilateral frontal hypometabolism was associated with severe developmental impairment. Two children with bitemporal hypometabolism exhibited autistic features. Hemiparesis was associated with superior frontal (motor cortex) hypometabolism. Three patients underwent resective surgery, resulting in improved seizure control and developmental outcomes. The severity of neurologic complications and clinical course depend on the extent of cortical dysfunction in bilateral Sturge-Weber syndrome. Bilateral frontal and temporal hypometabolism is associated with poor developmental outcomes. Good seizure control and only mild/moderate developmental impairment can be achieved in about 50% of patients with bilateral Sturge-Weber syndrome, with or without resective surgery.
authors.affiliation: Carman and Ann Adams Department of Pediatrics, Wayne State University School of Medicine, Detroit, Michigan 48201, USA.
authors.forename: Bálint
authors.initials: B
authors.lastname: Alkonyi
authors.forename: Harry T
authors.initials: HT
authors.lastname: Chugani
authors.forename: Samir
authors.initials: S
authors.lastname: Karia
authors.forename: Michael E
authors.initials: ME
authors.lastname: Behen
authors.forename: Csaba
authors.initials: C
authors.lastname: Juhász
mesh_terms: D000293:Adolescent; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D049268:Positron-Emission Tomography / Q000379:methods; D012189:Retrospective Studies; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000378:metabolism*; D016896:Treatment Outcome; D055815:Young Adult