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Early infantile epileptic encephalopathy

Disease definition

A severe form of age-related epileptic encephalopathies characterized by the onset of tonic spasms within the first 3 months of life that can be generalized or lateralized, independent of the sleep cycle, and that can occur hundreds of times per day, leading to psychomotor impairment and death.


Classification level: Disorder
  • Synonym(s):
    • EIEE
    • Early infantile epileptic encephalopathy with suppression-bursts
    • Ohtahara syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or Autosomal recessive or X-linked recessive or Not applicable 
  • Age of onset: Neonatal
  • ICD-10: G40.3
  • OMIM: 300672  308350  609304  612164  613402  613721  615473  616341  617276  617350  617389  617391  617493  617599
  • UMLS: C0393706
  • MeSH: -
  • GARD: 9255
  • MedDRA: 10071545

Detailed information


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