Long-term course of West syndrome associated with tuberous sclerosis.
We retrospectively analyzed the long-term clinical profiles of 47 patients with West syndrome (WS) associated with tuberous sclerosis (TS) the follow-up study for >10 years showed that WS developed into epilepsies of various types in all patients. Their final diagnoses were symptomatic generalized epilepsy (SGE: 29 patients, 62%), symptomatic partial epilepsy (PE: 14 patients, 30%), and undetermined epilepsy (UE: 4 patients, 8%). Eighty-five percent of all patients continued to show the same types of epilepsy that they had at their first examination, but in 15% the diagnosis had to be revised at the end of the follow-up period. The outcome of seizures was unfavorable for the SGE group. In the PE group, however, seizures were controlled in 9 (64%) of the 14 patients, and disturbances of daily activity were mild. In patients with WS associated with TS, the seizure outcome and social activity varied depending on the type of epilepsy or epileptic syndrome that developed from WS. The complicated clinical developments from WS with TS multiple cortical tubers related to epileptogenesis in addition to impairment of brain development.
Fukushima
K
K
National Epilepsy Center, Shizuoka Higashi Hospital, Urushiyama, Japan.
Inoue
Y
Y
Fujiwara
T
T
Yagi
K
K
D000293:Adolescent; D000367:Age Factors; D001921:Brain / Q000000981:diagnostic imaging / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000706:statistics & numerical data; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D017063:Outcome Assessment, Health Care; D011379:Prognosis; D012189:Retrospective Studies; D012640:Seizures / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D013036:Spasms, Infantile / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology