Effect of adrenocorticotropic hormone therapy for epileptic spasms developing after the age of 1 year.
OBJECTIVE
Epileptic spasms sometimes begin after the first year of life, and such seizures are recognized as late-onset spasms (LOS). The prognosis of LOS is poor, and a treatment strategy has not been established. This study aimed to assess the short- and long-term effects of adrenocorticotropic hormone (ACTH) therapy for LOS.


METHODS
We investigated the rate of LOS in 22 patients (14 boys and 8 girls) treated with ACTH therapy. The age at onset of LOS and at the start of ACTH therapy ranged from 12 to 94 months (median, 31.6 ± 22.1 months) and from 12.5 to 116 months (median, 37.5 ± 23.7 months), respectively. We investigated the response rate of LOS treated with ACTH therapy, and compared the clinical features between responders (short-term) and nonresponders.


RESULTS
Nine (41%) of the 22 patients showed cessation of epileptic spasms within 3 months. The epileptic spasms ceased in four of these nine patients for more than 1 year. The age at onset of LOS was significantly associated with short-term seizure cessation (p<0.05). Patients who achieved short-term cessation of seizures received ACTH therapy within 6 months from the onset of LOS.


CONCLUSIONS
ACTH therapy is a potentially effective treatment when started within 6 months from the onset of LOS. A younger age at onset of LOS is associated with a favorable outcome.
Department of Pediatrics, Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka 569-8686, Japan.
Miho
M
Fukui
Department of Pediatrics, Osaka Medical College, 2-7 Daigaku-machi, Takatsuki, Osaka 569-8686, Japan. Electronic address: ZVQ10523@nifty.com.
Shuichi
S
Shimakawa
Department of Child Neurology, Osaka City General Hospital, 2-15-16, Miyakojima Hon-Dori, Miyakojima-ku, Osaka 534-0021, Japan.
Ichiro
I
Kuki
Department of Child Neurology, Osaka City General Hospital, 2-15-16, Miyakojima Hon-Dori, Miyakojima-ku, Osaka 534-0021, Japan.
Hisashi
H
Kawawaki
Department of Pediatric Neurology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodocho Izumi, Osaka 594-1101, Japan.
Yukiko
Y
Mogami
Department of Pediatric Neurology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodocho Izumi, Osaka 594-1101, Japan.
Yasuhiro
Y
Suzuki
Department of Pediatrics, Osaka University Graduate School of Medicine, 2-15 Yamadaoka Suita, Osaka 565-0871, Japan.
Sayaka
S
Nakano
Department of Pediatrics, Osaka University Graduate School of Medicine, 2-15 Yamadaoka Suita, Osaka 565-0871, Japan.
Takeshi
T
Okinaga
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D000367:Age Factors; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006728:Hormones / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000188:drug therapy*; D016896:Treatment Outcome
D006728:Hormones; D000324:Adrenocorticotropic Hormone
ACTH; Late-onset infantile spasms; Outcome; Treatment; Treatment lag