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Distal Myopathy

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Professor Nigel G. Laing, NH & MRC Principal Research Fellow, Centre for Medical Research, University of Western Australia, West Australian Institute for Medical Research, for assistance in the preparation of this report.

Synonyms of Distal Myopathy

  • Distal Muscular Dystrophy

Disorder Subdivisions

  • Distal Myopathy 3 (MPD3)
  • Distal Myopathy with Rimmed Vacuoles (DMRV)
  • Distal Myopathy with Vocal Cord and Pharyngeal Signs
  • Inclusion Body Myopathy Type 2 (IBM2)
  • Laing Distal Myopathy
  • Laing Early-Onset Distal Myopathy
  • Miyoshi Myopathy
  • Nonaka Myopathy
  • Tibial Distal Myopathy
  • Udd Distal Myopathy
  • Welander Distal Myopathy

General Discussion

Distal myopathy (or distal muscular dystrophy) is a general term for a group of rare progressive genetic disorders characterized by wasting (atrophy) and weakness of the voluntary distal muscles. The distal muscles are those farther from the center of the body and include the muscles of the lower arms and legs and the hands and feet. Conversely, the proximal muscles are the muscles closest to the center of the body such as the muscles of the shoulder, pelvis, and upper arms and legs. Although age of onset can occur anytime from infancy to adulthood, most forms develop later in life and are slowly progressive. Inheritance is autosomal dominant or recessive.

The distal myopathies belong to a larger group of disorders known as the muscular dystrophies. The muscular dystrophies are characterized by weakness and degeneration of various voluntary muscles of the body. Approximately 30 different disorders make up the muscular dystrophies. The disorders affect different muscles and have different ages of onset, severity and inheritance patterns.

Organizations related to Distal Myopathy

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder. .

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