Orphanet: Late onset distal myopathy, Markesbery Griggs type

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Late-onset distal myopathy, Markesbery-Griggs type

Disease definition

A rare, genetic, non-dystrophic myofibrillar myopathy disorder characterized by late-adult onset of distal and/or proximal limb muscle weakness with initial involvement of posterior lower leg muscles, medial gastrocnemius and soleus. Patients present with ankle weakness followed by weakness of finger and wrist extensors and later on of proximal muscles. Ambulation is usually preserved. Late-onset associated cardiomyopathy and/or neuropathy has been reported in a minority of cases.


Classification level: Disorder
  • Synonym(s):
    • ZASP-related myofibrillar myopathy
  • Prevalence: <1 / 1 000 000
  • Inheritance: -
  • Age of onset: Adult
  • ICD-10: G71.8
  • OMIM: 609452
  • UMLS: C1836155
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

General public

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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