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Dentatorubral pallidoluysian atrophy

Disease definition

Dentatorubral pallidoluysian atrophy (DRPLA) is a rare subtype of type I autosomal dominant cerebellar ataxia (ADCA type I; see this term). It is characterized by involuntary movements, ataxia, epilepsy, mental disorders, cognitive decline and prominent anticipation.

ORPHA:101

  • Synonym(s):
    • DRPLA
    • Dentatorubropallidoluysian atrophy
    • Naito-Oyanagi disease
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: G11.8
  • OMIM: 125370
  • UMLS: C0751781
  • MeSH: -
  • GARD: 5643
  • MedDRA: -

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