Researchers at Wayne State University School of Medicine, Detroit, MI studied 14 children with bilateral Sturge-Weber syndrome, representing 12.7% of a total of 110 patients with the disorder. Glucose positron emission tomography had an asymmetric pattern, one hemisphere was hypometabolic in 3 patients. Signs of cerebral atrophy or vascular abnormalities on MRI were more pronounced on one side. Long-term follow-up data available for 8 of the 14 patients covered 1-16 years (median, 9 years). Age at onset of seizures in 14 patients ranged from 2 weeks to 7.1 years (median, 6 months). Seizure type was mostly complex partial, and seizure control was satisfactory in 6 (47%). Patients with poor seizure control manifested frontal glucose hypometabolism on PET. Nine patients (64%) had hemiparesis, with contralateral frontal hypometabolism. Bilateral frontal and temporal hypometabolism was coupled with severe developmental impairment. No patient with good seizure control had severe developmental impairment. Developmental impairment was not related to age at onset of first seizure. Autism in 2 patients was associated with bitemporal hypometabolism on PET. Three patients who underwent epilepsy surgery had complete remission of refractory seizures but development was only partially improved. With or without resective surgery, good seizure control is achieved in about 50% of patients with bilateral Sturge-Weber syndrome. [1]
COMMENT. PET studies are important in the evaluation of children with bilateral Sturge-Weber syndrome as follows: 1) prediction of outcome, seizure control, and impairment of development and cognition; 2) risk of autism related to bitemporal hypometabolism; and 3) determination of functional integrity of the nonresected hemisphere before surgery. Early resection of a hemisphere or focus may be beneficial in some bilateral cases with intractable seizures. Persistent seizures may contribute to neurocognitive decline.