Surgical treatment of Sturge-Weber syndrome--case report

It is well known that when Sturge-Weber syndrome manifests with seizures in early infancy, hemiparesis develops early, seizures become intractable, and motor weakness and mental retardation are progressive. In North America and Europe, early surgical intervention is recommended in such cases. However, neurosurgical management of Sturge-Weber syndrome has not been reported in Japan. The authors describe a 4-month-old boy with Sturge-Weber syndrome accompanied by intractable seizures who was successfully treated by a two-stage hemispherectomy. Two years postoperatively he remains free of seizures and is active, although his psychomotor development is moderately retarded. Surgical treatment of Sturge-Weber syndrome, including long-term results, is discussed in detail.