Article

Treatment Outcomes of West Syndrome in Infants With Down Syndrome

Saul R. Korey Department of Neurology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York. Electronic address: .
Pediatric Neurology (Impact Factor: 1.7). 01/2013; 48(1):42-7. DOI: 10.1016/j.pediatrneurol.2012.09.006
Source: PubMed

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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    • "Thereafter, spasms tend to disappear before 5 years of age, but relapses are possible, as in our patient. The long-term follow-up showed that 60% of the children had drug-resistant epilepsy and that 75% of the children had a delay in their psychomotor development [18] [19]. It is generally accepted that children with West syndrome who have evidence of pre-existing developmental delay or neurological abnormalities have a worse prognosis with a poorer response to treatment and less favorable developmental outcome [4]. "
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