Central nervous system structure and function in Sturge-Weber syndrome: evidence of neurologic and radiologic progression.
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.
Maria
B L
BL
Department of Pediatrics, University of Florida College of Medicine, Gainesville, USA.
Neufeld
J A
JA
Rosainz
L C
LC
Drane
W E
WE
Quisling
R G
RG
Ben-David
K
K
Hamed
L M
LM
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001241:Aspirin / Q000627:therapeutic use; D001921:Brain / Q000098:blood supply* / Q000000981:diagnostic imaging / Q000473:pathology; D002561:Cerebrovascular Disorders / Q000517:prevention & control; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005947:Glucose / Q000378:metabolism*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012640:Seizures / Q000503:physiopathology; D013341:Sturge-Weber Syndrome / Q000150:complications / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed
D000894:Anti-Inflammatory Agents, Non-Steroidal; D005947:Glucose; D001241:Aspirin