Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression

• Published in: Journal of child neurology Vol. 13; no. 12; p. 606
• Main Authors: Maria, B L, Neufeld, J A, Rosainz, L C, Drane, W E, Quisling, R G, Ben-David, K, Hamed, L M
• Format: Journal Article
• Language: English
• Published: United States 01-12-1998
• Subjects: Adolescent; Adult; Age of Onset; Anti-Inflammatory Agents, Non-Steroidal - therapeutic use; Aspirin - therapeutic use; Brain - blood supply; Brain - diagnostic imaging; Brain - pathology; Cerebrovascular Disorders - prevention & control; Child; Child, Preschool; Female; Follow-Up Studies; Glucose - metabolism; Humans; Infant; Magnetic Resonance Imaging; Male; Seizures - physiopathology; Sturge-Weber Syndrome - complications; Sturge-Weber Syndrome - physiopathology; Tomography, X-Ray Computed
• ISSN: 0883-0738
• DOI: 10.1177/088307389801301204

Sturge-Weber syndrome is characterized by the presence of a port-wine nevus, epilepsy, stroke-like episodes, headache, and developmental delay. We studied 20 cases to test the hypothesis that decreased cerebral blood flow alters neurologic function by affecting cellular glucose metabolism. Group A consisted of 10 patients with a mean age of 1.75 years and early seizure onset (6.8 months), whereas group B was composed of older patients (mean age, 15.3 years) with later onset of seizures (3.7 years). Neurologic disease was more severe in group A, but group B had more widespread structural brain defects - shown on computed tomographic scans and magnetic resonance imaging - and metabolic brain defects shown on hexamethylpropyleneamine oxime and [18F] fluorodeoxyglucose single photon emission computed tomographic scans. Six group A cases had hypoperfusion at baseline and five of nine had worsening of perfusion and glucose metabolism 1 year later. A total of 119 stroke-like episodes occurred in six group A cases and eight group B cases; there were 65% fewer strokes in children treated with aspirin. The data suggest that progressive hypoperfusion and glucose hypometabolism are associated with neurologic deterioration in Sturge-Weber syndrome. Longitudinal studies are needed to better define the natural history of disease and to evaluate the safety and efficacy of aspirin therapy.