The idiopathic form of West syndrome

Epilepsia. 1993 Jul-Aug;34(4):743-6. doi: 10.1111/j.1528-1157.1993.tb00456.x.

Abstract

The clinical and electroencephalographic data of 31 children with cryptogenic West syndrome (WS), selected from a series of 103 WS patients, with a follow-up between 4 and 12 years, were studied retrospectively to verify whether this group included patients who fulfilled the criteria for an idiopathic etiology. The results identified a possible idiopathic etiology in 17 patients (55%), who had a family history of other forms of idiopathic epilepsy or febrile convulsions, or who developed, during the follow-up, an EEG genetic trait such as a photoconvulsive response or spike-and-wave discharges, or rolandic spikes. All 17 children had a favorable outcome and all had normal neuropsychological development. Four children (13%) fulfilled the criteria for a true cryptogenic etiology, a causative lesion being suspected, but never proved. At the end of the follow-up all four had seizures, or developmental delay or both, all signs that suggest an underlying cerebral lesion. The other 10 children, representing 32% of the cryptogenic cases, had a good prognosis, with early disappearance of spasms and hypsarrhythmia, and normal neurological development, but none had an EEG epileptic trait or family history of epilepsy or febrile convulsions; although they could have had an idiopathic WS, this was not proved. We conclude that among the children classified as having a cryptogenic WS, many--in our series at least 55%--fulfill the criteria for an idiopathic etiology.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Electroencephalography
  • Epilepsy / classification
  • Epilepsy / diagnosis
  • Epilepsy / genetics
  • Family
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Male
  • Prognosis
  • Retrospective Studies
  • Seizures, Febrile / diagnosis
  • Seizures, Febrile / genetics
  • Spasms, Infantile / classification
  • Spasms, Infantile / diagnosis*
  • Spasms, Infantile / genetics