Orphanet: Steinert myotonic dystrophy

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Steinert myotonic dystrophy

Disease definition

Steinert disease, also known as myotonic dystrophy type 1, is a muscle disease characterized by myotonia and by multiorgan damage that combines various degrees of muscle weakness, arrhythmia and/or cardiac conduction disorders, cataract, endocrine damage, sleep disorders and baldness.


Classification level: Disorder
  • Synonym(s):
    • DM1
    • MD1
    • Myotonic dystrophy type 1
    • Steinert disease
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Neonatal, Adult
  • ICD-10: G71.1
  • OMIM: 160900
  • UMLS: C2931688
  • MeSH: C538008
  • GARD: 8310
  • MedDRA: -
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