Orphanet: Encefalopatia mioclónica precoce

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Early myoclonic encephalopathy

Disease definition

A rare disorder characterized clinically by the onset of fragmentary myoclonus appearing in the first month of life, often associated with erratic focal seizures and a suppression-burst EEG pattern.


Classification level: Disorder
  • Synonym(s):
    • Early myoclonic encephalopathy with suppression-bursts
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive or Autosomal dominant 
  • Age of onset: Neonatal, Infancy
  • ICD-10: G40.4
  • OMIM: 609304  616341  617105
  • UMLS: C0270855
  • MeSH: -
  • GARD: -
  • MedDRA: -
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