Outcome of infants with unilateral Sturge-Weber syndrome and early onset seizures

Patients with Sturge-Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant se izures who do not respond to medical treatment are candidates for early epi leptic surgery, However, a delay of surgical treatment may result in cognit ive deterioration. We studied the correlation between parameters and outcom e of seizures to re-examine the criteria for early epilepsy surgery, We per formed a retrospective chart review combined with telephone interviews of p arents of all Israeli infants with unilateral Sturge-Weber syndrome and ear ly onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemipares is at follow-up. We recruited a total of 15 patients with unilateral Sturge -Weber syndrome and early onset seizures, five of whom underwent epilepsy s urgery. The mean follow-up period of all the patients was 15 years: six pat ients had normal intelligence, four had borderline cognitive level, three h ad mild mental retardation* and two had moderate mental retardation, Eight of the ten non-operated patients still experience seizures at follow-up, co gnitive delay was significantly correlated with seizure intensity in the ea rly period, but not with the age of seizures onset, the degree of hemipares is, or the presence of ongoing seizures. We conclude that high seizure inte nsity in young patients with Sturge-Weber syndrome is a prognostic marker f or mental deterioration.