Long-term follow-up study of West syndrome associated with tuberous sclerosis

Aim: To analyze the evolution of seizures, electroencephalographic (EEG) features, seizure outcomes, and social outcomes, in order to formulate a basis for the long-term prognosis of West syndrome (WS) associated with tuberous sclerosis (TS). Method: The clinical profiles of 50 patients with TS, who presented with WS in infancy and had been monitored for more than 10 years after the initial examination, were analyzed. Results: In 86% of the patients the seizure type did not change throughout the course of this study. However, 14% of patients had developed other types of seizures by the end of the follow-up period. The seizure outcome was unfavorable for patients with generalized seizures. Partial seizures were controlled in nine (64%) of the 14 patients, and normal or slightly subnormal intelligence accounted for seven of the 14 patients. Conclusions: Therefore, in patients with WS associated with TS, the seizure outcome and social activity depended on the type of seizure that developed during the course of this disorder. The outcome of patients with WS associated with TS seems to be better than that of WS in general.