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Autosomal dominant limb-girdle muscular dystrophy type 1B

Disease definition

A rare subtype of autosomal dominant limb girdle muscular dystrophy characterized by a variable age of onset of progressive shoulder and hip girdle weakness, with inferior limbs usually being affected prior to upper limbs, and mild joint contractures. It is also associated with cardiac dysrhythmias, including atrioventricular conduction blocks, and late-onset dilated cardiomyopathy, that may lead to sudden death.


Classification level: Disorder
  • Synonym(s):
    • LGMD1B
    • Limb-girdle muscular dystrophy due to lamin A/C deficiency
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Childhood
  • ICD-10: G71.0
  • OMIM: 159001  181350
  • UMLS: C1834653
  • MeSH: -
  • GARD: 10230
  • MedDRA: -

Detailed information

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