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Published on: November 21, 2013

West syndrome: early remission is not assurance of normal final outcome

A P de Queiroz Campos Araújo1, L M da Costa Fontenelle, L Pires

  • 1The Federal University of Rio de Janeiro, Rio de Janeiro, Brazil. alexprufer@ufrj.br

Child: Care, Health and Development
|July 20, 2010
PubMed

Insights

West syndrome (WS) is an infant epilepsy that may remit. However, early seizure remission in WS does not guarantee normal long-term development or seizure freedom in children.

Area of Science:

  • Pediatric Neurology
  • Epileptology

Background:

  • West syndrome (WS) is an age-specific epilepsy in infancy and early childhood.
  • WS is generally considered to have a poor prognosis, though remission is possible.

Purpose of the Study:

  • To describe the clinical features, treatment, and outcomes of children with WS in a Latin American country.
  • To analyze short-term and long-term outcomes in infants diagnosed with WS.

Main Methods:

  • Retrospective study of 37 infants diagnosed with WS.
  • Included infants with recurrent spasms and hypsarrhythmia on EEG.
  • Reviewed medical records focusing on anti-epileptic drugs, steroids, and developmental outcomes.

Main Results:

  • Most cases (78.3%) were symptomatic, with neonatal hypoxia being the most frequent etiology.
  • A favorable short-term outcome (spasm remission up to 6 months) was observed in 20 infants.
  • Only three infants achieved normal development and remained seizure-free after age 2.

Conclusions:

  • Early seizure remission in West syndrome does not predict a normal final outcome.
  • Long-term neurodevelopmental status and seizure control remain significant challenges in WS.
Abstract

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