Abstracts

Rationale: To evaluate the seizure evolution and clinical outcome in patients with West syndrome (WS) associated with tuberous sclerosis (TS). Methods: Sixteen patients diagnosed as having WS with TS from 1990 to 2002 at Seoul National University Children’s Hospital were enrolled. We followed them over at least 5 years from spasm onset and analyzed their clinical features focusing on remission of spasm, seizure evolution, and final outcome of seizure control. Vigabatrin was tried as initial treatment of spasm after 1996 in place of ACTH or prednisolone in 7 patients. Results: The average onset of spasm was 6.8 months (3.6 - 10.6 months). Two patients showed only infantile spasms throughout their clinical courses. Other type of seizures were developed in 14 patients (88%) at the average age of 29 months (4.4-71 months). Half of them showed partial seizures as the predominant seizure type, and the other half showed mixed features of both generalized and partial seizures. Among 16 patents, 12 patients (75 %) had remission of spasm with vigabatrin, ACTH, steroid, and ketogenic diet(KD). As for long-term seizure outcome, 75 % (12/16) of patients maintained remission of seizures at least for 1 year. Three patients with intractable courses had common features of poorly controlled spasms with ACTH or steroid, and their seizures evolved to the combination of generalized and partial seizures in the later period. In 6 patients with good seizure control, antiepileptic drugs were discontinued, however, 2 of them experienced seizure relapse. Seven patients initially treated with vigabatrin acquired complete control of spasms, and evolved to partial seizures as the main seizure type. Their partial seizures were well controlled with combination of AED and/or KD and/or surgery except one patient who had seizure relapse after 4 years of seizure remission. Conclusions: Excellent control of spasms in WS with TS seems to play an important role for limiting the later seizure types as partial seizure. It would also result in favorable long-term seizure outcome as well.