Multicenter long-term follow-up of children with idiopathic West syndrome: ACTH versus vigabatrin.
OBJECTIVE
Long-term follow-up of children with idiopathic West syndrome (WS) treated with adrenocorticotropic hormone (ACTH) or vigabatrin.


METHODS
Records of 28 normal magnetic resonance imaging (MRI) WS cases were reviewed for seizure development and cognitive outcome in relation to treatment type and lag.


RESULTS
Average age at disease onset was 5.5 months, and average lag time to treatment was 25 days. Fourteen patients were treated with ACTH (eight early and six late), and 14 with vigabatrin (without delay). Response rates were 88% for ACTH and 80% for vigabatrin. Short-term outcomes for seizure cessation and electroencephalography normalization were identical between the groups. In the long-term, early ACTH treatment was better than the rest combined. Average follow-up time was 9 years. A normal cognitive outcome was achieved in 100% of the early-ACTH group, 67% of the late-ACTH group and 54% of the vigabatrin group (P = 0.03). Seizures subsequently developed in 54% of the vigabatrin group, in 33% of the late ACTH group, and 0% of the early ACTH group (P < 0.05).


CONCLUSIONS
Idiopathic WS with normal MRI is associated with a good cognitive outcome. Early ACTH treatment, administered within 1 month, yields a better cognitive and seizure outcome than vigabatrin or late ACTH.
Cohen-Sadan
S
S
Pediatric Neurology Unit, Dana Children's Hospital, Tel Aviv, Israel.
Kramer
U
U
Ben-Zeev
B
B
Lahat
E
E
Sahar
E
E
Nevo
Y
Y
Eidlitz
T
T
Zeharia
A
A
Kivity
S
S
Goldberg-Stern
H
H
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use*; D001921:Brain / Q000187:drug effects / Q000503:physiopathology; D002648:Child; D002657:Child Development / Q000187:drug effects*; D002675:Child, Preschool; D003071:Cognition / Q000187:drug effects; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012640:Seizures / Q000188:drug therapy; D013036:Spasms, Infantile / Q000188:drug therapy*; D016896:Treatment Outcome; D020888:Vigabatrin / Q000627:therapeutic use*
D000927:Anticonvulsants; D000324:Adrenocorticotropic Hormone; D020888:Vigabatrin