title: West syndrome in tuberous sclerosis complex.
abstract: West syndrome occurs commonly in children with tuberous sclerosis complex and is associated with a grave prognosis for cognitive and seizure outcomes. We sought to determine the epilepsy outcome of children with tuberous sclerosis complex and West syndrome and whether EEG, MRI, or steroid therapy duration were different in those whose epilepsy improved compared with those with intractable seizures. Seventeen patients with tuberous sclerosis complex and West syndrome were identified. For each patient, two sets of clinical evaluations, EEG and MRI data, and treatment information separated by at least 12 months were obtained. The patients were divided into two seizure outcome groups. EEG, MRI, and treatment data were compared between the groups. The intellectual deficiency was either severe (76%) or moderate (24%). Seizure control improved in 10 and worsened in seven, without mortality (follow-up range = 12-216 months). No significant differences in EEG background, MRI findings, or steroid treatment duration were evident between the groups. The difference in EEG-sleep approached statistical significance (P = 0.06). Our findings did not confirm reports of high mortality and poor epilepsy outcome in intellectually deficient children with West syndrome and tuberous sclerosis complex. EEG sleep was the best indicator of seizure control and approached statistical significance. The duration of steroid therapy had no influence on seizure control.
authors.affiliation: Department of Medicine, Division of Neurology, Duke University and Neurodiagnostic Center, Veterans Affairs Medical Center, Durham, North Carolina 27710, USA.
authors.forename: A M
authors.initials: AM
authors.lastname: Husain
authors.forename: C M
authors.initials: CM
authors.lastname: Foley
authors.forename: A
authors.initials: A
authors.lastname: Legido
authors.forename: D A
authors.initials: DA
authors.lastname: Chandler
authors.forename: D K
authors.initials: DK
authors.lastname: Miles
authors.forename: W D
authors.initials: WD
authors.lastname: Grover
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000927:Anticonvulsants / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D015996:Survival Rate; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality
chemical_list: D000305:Adrenal Cortex Hormones; D000927:Anticonvulsants