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Early infantile epileptic encephalopathy

Orpha number ORPHA1934
Synonym(s) EIEE
Early infantile epileptic encephalopathy with suppression-bursts
Ohtahara syndrome
Prevalence <1 / 1 000 000
Inheritance
  • Sporadic
  • Autosomal dominant
  • Autosomal recessive
  • X-linked recessive
Age of onset Neonatal/infancy
ICD-10
  • G40.3
OMIM
UMLS -
MeSH -
MedDRA -
SNOMED CT
  • 230429005

Summary

Early infantile epileptic encephalopathy (EIEE) or Ohtahara syndrome is the most severe form of age-related epileptic encephalopathy and characterized by the onset of tonic spasm 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.


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