Treatment outcomes of west syndrome in infants with down syndrome

Oranee Sanmaneechai, Yoshimi Sogawa, Wendy Silver, Karen R. Ballaban-Gil, Solomon L. Moshe, Shlomo Shinnar

Research output: Contribution to journalArticle

19 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)42-47
Number of pages6
JournalPediatric Neurology
Volume48
Issue number1
DOIs
StatePublished - Jan 2013

Fingerprint

Infantile Spasms
Down Syndrome
Seizures
Clonazepam
Recurrence
Autistic Disorder
Intellectual Disability
Adrenocorticotropic Hormone
Electroencephalography
Epilepsy
Parents
Databases
Therapeutics

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Treatment outcomes of west syndrome in infants with down syndrome. / Sanmaneechai, Oranee; Sogawa, Yoshimi; Silver, Wendy; Ballaban-Gil, Karen R.; Moshe, Solomon L.; Shinnar, Shlomo.

In: Pediatric Neurology, Vol. 48, No. 1, 01.2013, p. 42-47.

Research output: Contribution to journalArticle

Sanmaneechai, Oranee ; Sogawa, Yoshimi ; Silver, Wendy ; Ballaban-Gil, Karen R. ; Moshe, Solomon L. ; Shinnar, Shlomo. / Treatment outcomes of west syndrome in infants with down syndrome. In: Pediatric Neurology. 2013 ; Vol. 48, No. 1. pp. 42-47.
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