Prognosis in Sturge-Weber disease: comparison of unihemispheric and bihemispheric involvement

One hundred two patients with Sturge-Weber disease who were seen at the Mayo Clinic between 1942 and 1986 were studied retrospectively to determine the difference in prognosis between unihemispheric (88 patients) and bihemispheric (14 patients) involvement. Seizures occurred in 63 with unihemispheric involvement and 13 with bihemispheric; the mean age at onset of seizures was 24 months in the former and 6 months in the latter. Of the total group, 19% were severely or moderately mentally retarded, 27% were mentally retarded but educable, and 45% had average intelligence. In the bihemispheric involvement group, 46% were severely or moderately mentally retarded, 38% were retarded but educable, and only 8% had average intelligence. Bilateral involvement of the brain by Sturge-Weber disease is associated with earlier onset of seizures and worse prognosis for mental development compared with unilateral involvement.