8C71.0

ICD-11 MMS 2026-01

Myotonic dystrophy

Description

Myotonic dystrophy is a group of inherited muscular disorders. It is the most common form of muscular dystrophy that begins in adulthood. Myotonic dystrophy is characterised by progressive muscle wasting and weakness, and prolonged muscle contractions (myotonia) that are not able to relax after use. Other signs and symptoms of myotonic dystrophy include slurred speech or temporary locking of their jaw, cataracts and cardiac conduction defects. In affected men, hormonal changes may lead to early balding and infertility. The clinical severity varies widely among affected patients, even among members of the same family.

Index terms

  • Myotonic dystrophy
  • DM - [dystrophia myotonica]
  • dystrophia myotonica
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  • myotonia atrophica
  • myotonia dystrophica
  • myotonic muscular dystrophy
  • Myotonic dystrophy type 1
  • DM1 - [Myotonic dystrophy type 1]
  • Steinert disease
  • Steinert myotonic dystrophy syndrome
  • Steinert syndrome
  • Congenital myotonic dystrophy
  • CDM - (congenital myotonic dystrophy)
  • Juvenile to adult onset myotonic dystrophy
  • Myotonic dystrophy type 2
  • PROMM - [proximal myotonic myopathy]

Excludes

  • http://id.who.int/icd/entity/1159616108 – Secondary myopathies
  • http://id.who.int/icd/entity/1425591047 – Myasthenia gravis or certain specified neuromuscular junction disorders

Exclusions from above levels

  • http://id.who.int/icd/entity/155258022 – Metabolic disorders
  • LD26.41 – Arthrogryposis multiplex congenita
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8C71.1 – Chondrodystrophic myotonia
  • 8C71.2 – Myotonia congenita
  • 8C71.3 – Drug-induced myotonia
  • 8C71.4 – Neuromyotonia
  • 8C71.5 – Pseudomyotonia
  • 8C71.Y – Other specified myotonic disorders
  • 8C71.Z – Myotonic disorders, unspecified

Foundation entity

Myotonic dystrophy – http://id.who.int/icd/entity/192087511

Part of: 8C71: Myotonic disorders › 08: Diseases of the nervous system