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West syndrome

Disease definition

West syndrome (or infantile spasms) is characterised by the association of clusters of axial spasms, psychomotor retardation and an hypsarrhythmic interictal EEG pattern. It is the most frequent type of epileptic encephalopathy. It may occur in otherwise healthy infants and in those with abnormal cognitive development.

ORPHA:3451

  • Synonym(s):
    • Infantile spasms
    • Intellectual disability-hypsarrhythmia syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant or Autosomal recessive or X-linked recessive 
  • Age of onset: Infancy, Neonatal, Childhood
  • ICD-10: G40.4
  • OMIM: 300672  308350  613477  613722  615006  616139  616341  617065
  • UMLS: C0037769
  • MeSH: -
  • GARD: 7887
  • MedDRA: 10021750

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