Surgical outcome in tuberous sclerosis complex: a multicenter survey.

Summary: Multicenter, retrospective analysis of 70 subjectswith TSC following surgery for relief of epilepsy revealedsignificant associations between younger age at seizure onset,present/prior history of infantile spasms, interictal focality (bi-lateral versus unilateral), and absence of residual postoperativepredominanttuber,andpoorerpostoperativeoutcome(p < 0.01).Ictal multifocality, mental retardation, and discordant EEG andMRI data showed a negative trend toward outcome, but werenot significant. KeyWords: Tuberous sclerosis—Surgical—Outcome. Epilepsy often complicates tuberous sclerosis complex(TSC). Cortical tubers are pathognomic of TSC and con-tribute to the pathogenesis of seizures. Depending on theseizure type, ictal EEG can display focal or generalizedseizures, including infantile spasms. Focal features oftenreflect the predominant epileptogenic tuber topography.Seizures in TSC are often resistant to antiepileptic drug(AED) therapy, but surgery may control seizures in se-lected cases. Outcome is best in patients with a singleseizure type, single tuber, or one large tuber, with con-vergent clinical, imaging and neurophysiologic data (Ro-manelli et al., 2004), and no mental retardation (Jarraret al., 2004). Current techniques allow for better localiza-tion of epileptogenic tissue, leading to improved surgicaloutcome (Weiner, 2004; Weiner et al., 2004).