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Monomelic amyotrophy

Disease definition

Monomelic amyotrophy (MA) is a rare benign lower motor neuron disorder characterized by muscular weakness and wasting in the distal upper extremities during adolescence followed by a spontaneous halt in progression and a stabilization of symptoms.

ORPHA:65684

  • Synonym(s):
    • Benign focal amyotrophy
    • Hirayama disease
    • JMADUE
    • Juvenile muscular atrophy of distal upper extremity
    • Juvenile muscular atrophy of the distal upper limb
  • Prevalence: Unknown
  • Inheritance: Unknown 
  • Age of onset: Adolescent, Adult
  • ICD-10: G12.8
  • OMIM: 602440
  • UMLS: C1865384
  • MeSH: C538253
  • GARD: 9697
  • MedDRA: 10069681

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