title: Treatment outcomes of West syndrome in infants with Down syndrome.
abstract: West syndrome constitutes the most frequent of all seizure types in infants with Down syndrome. We retrospectively reviewed records of 12 infants with Down syndrome and West syndrome, accounting for 5% of 239 infants with West syndrome from a comprehensive epilepsy database during a 17-year period. All demonstrated classic hypsarrhythmia on video electroencephalograms. One had clinically responded to clonazepam, and one was not treated because the parents refused any treatment. Seven of 10 infants demonstrated a complete response to high-dose natural adrenocorticotrophic hormone. Four (57%) of these seven infants relapsed. Relapses occurred as long as 2 years after cessation of the initial presentation of infantile spasms. At most recent follow-up (median age, 5 years), 8/12 (67%) were seizure-free, and seven were off any medications. Two of three nonresponders manifested intractable epilepsy and profound mental retardation. Developmentally, 6/8 who could be assessed met criteria for autistic spectrum disorder. Close follow-up is necessary even after successful initial treatment, because relapses are frequent and can occur as long as 2 years later.
authors.affiliation: Saul R. Korey Department of Neurology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA. oranee141@gmail.com
authors.forename: Oranee
authors.initials: O
authors.lastname: Sanmaneechai
authors.forename: Yoshimi
authors.initials: Y
authors.lastname: Sogawa
authors.forename: Wendy
authors.initials: W
authors.lastname: Silver
authors.forename: Karen
authors.initials: K
authors.lastname: Ballaban-Gil
authors.forename: Solomon L
authors.initials: SL
authors.lastname: Moshé
authors.forename: Shlomo
authors.initials: S
authors.lastname: Shinnar
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D000927:Anticonvulsants / Q000627:therapeutic use*; D003072:Cognition Disorders / Q000209:etiology / Q000628:therapy; D016208:Databases, Factual / Q000706:statistics & numerical data; D004314:Down Syndrome / Q000150:complications*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D017063:Outcome Assessment, Health Care; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000209:etiology* / Q000628:therapy*; D016896:Treatment Outcome; D014741:Video Recording
chemical_list: D000927:Anticonvulsants; D000324:Adrenocorticotropic Hormone