NYMC Faculty Publications

Treatment Outcomes of West Syndrome in Infants with Down Syndrome

Author Type(s)

Faculty

DOI

10.1016/j.pediatrneurol.2012.09.006

Journal Title

Pediatric Neurology

First Page

42

Last Page

47

Document Type

Article

Publication Date

1-1-2013

Department

Pediatrics

Second Department

Neurology

Keywords

Adrenocorticotropic Hormone, Anticonvulsants, Cognition Disorders, Databases, Factual, Down Syndrome, Electroencephalography, Female, Humans, Infant, Longitudinal Studies, Male, Neuropsychological Tests, Outcome Assessment, Health Care, Retrospective Studies, Spasms, Infantile, Treatment Outcome, Video Recording

Disciplines

Medicine and Health Sciences

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.

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