Orphanet: Distal hereditary motor neuropathy type 7

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Distal hereditary motor neuropathy type 7

Disease definition

A rare, slowly progressive genetic peripheral neuropathy characterized by distal atrophy and weakness affecting the upper limbs (with a predilection for the thenar eminence) and subsequently the lower limbs, associated with uni- or bilateral vocal cord paresis leading to hoarse voice and breathing difficulties, and facial weakness.


Classification level: Disorder
  • Synonym(s):
    • Distal spinal muscular atrophy with vocal cord paralysis
    • dHMN7
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Adolescent, Adult
  • ICD-10: G12.2
  • OMIM: 158580  607641
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

Article for general public


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