Early onset and focal spike discharges as indicators of poor prognosis for myoclonic-astatic epilepsy.
BACKGROUND
Myoclonic-astatic epilepsy (MAE) is an epileptic syndrome characterized by unique myoclonus, myoclonic-astatic, or astatic seizures in childhood. MAE prognosis vary from spontaneous remission to intractable seizures with profound mental retardation.


OBJECTIVE
Identifying early risk factors may optimize the treatment of children with MAE. Our hypothesis is early onset age and focal spike discharges on EEG indicate a poor MAE prognosis.


METHODS
Using the medical records of 9 children with MAE, we analyzed their clinical histories, EEG findings, and seizure symptoms. All patients were given follow-up observations/treatments by our department for at least 2 years after MAE onset.


RESULTS
Five of the patients were given favorable prognoses because their seizures disappeared within 2 years of onset; the other 4 received poor prognoses because their seizures continued more than 2 years. MAE onset in patient with refractory seizures was earlier than that in those with a favorable prognosis (7-24 months vs. 23-38 months). All the patients with refractory seizures showed moderate or severe mental retardation. Among the 5 patients with good prognosis, EEGs showed two with focal spike discharges and three with only generalized spike discharges. In contrast, all cases with a poor prognosis had focal spike discharges.


CONCLUSIONS
MAE onset in patients with refractory seizures occurs earlier than in those with favorable prognosis. Prognosis was excellent when EEG findings show no focal spike discharges. Both early seizure onset and the focal spike discharges associated with MAE are indicators of poor prognosis.
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Takahito
T
Inoue
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Yukiko
Y
Ihara
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Yuko
Y
Tomonoh
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Noriko
N
Nakamura
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Shinya
S
Ninomiya
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Takako
T
Fujita
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Hiroshi
H
Ideguchi
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan.
Sawa
S
Yasumoto
Department of Biochemistry, School of Medicine, Fukuoka University, Fukuoka, Japan.
Bo
B
Zhang
Department of Pediatrics, School of Medicine, Fukuoka University, Fukuoka, Japan. Electronic address: hirose@fukuoka-u.ac.jp.
Shinichi
S
Hirose
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001921:Brain / Q000503:physiopathology*; D002648:Child; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D011379:Prognosis; D012307:Risk Factors; D055815:Young Adult
Doose syndrome; Early onset; Focal spike discharges; Myoclonic-astatic epilepsy; Prognosis