West syndrome in tuberous sclerosis complex

West syndrome occurs commonly in children with tuberous sclerosis complex a nd is associated with a grave prognosis for cognitive and seizure outcomes. We sought to determine the epilepsy outcome of children with tuberous scle rosis complex and West syndrome and whether EEG, MRI, or steroid therapy du ration were different in those whose epilepsy improved compared with those with intractable seizures, Seventeen patients with tuberous sclerosis compl ex and West syndrome were identified. For each patient, two sets of clinica l evaluations, EEG and MRI data, and treatment information separated by at least 12 months were obtained. The patients were divided into two seizure o utcome groups; EEG, MRI, and treatment data were compared between the group s. The intellectual deficiency was either severe (76%) or moderate (24%). S eizure control improved in 10 and worsened in seven, without mortality (fol low-up range = 12-216 months). No significant differences in EEG background , MRI findings, or steroid treatment duration were evident between the grou ps. The difference in EEG-sleep approached statistical significance (P = 0. 06). Our findings did not confirm reports of high mortality and poor epilep sy outcome in intellectually deficient children with West syndrome and tube rous sclerosis complex, EEG sleep was the best indicator of seizure control and approached statistical significance. The duration of steroid therapy h ad no influence on seizure control. (C) 2000 by Elsevier Science Inc. All r ights reserved.