8C73

ICD-11 MMS 2026-01

Mitochondrial myopathies

Description

Mitochondrial myopathies are heterogeneous group of disorders caused by dysfunction of mitochondrial oxidative phosphorylation and can be classified according to the associated biochemical, genetic defects (in the mitochondrial DNA or in nuclear encoded proteins) or clinical phenotype. Exclude: defects of mitochondrial respiratory chain, Kearns-Sayre syndrome, myoclonic epilepsy with ragged red fibres (MERRF)

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

Child codes

  • 8C73.0 – Autosomal recessive cardiomyopathy or ophthalmoplegia
  • 8C73.1 – Neuropathy, ataxia, and retinitis pigmentosa
  • 8C73.Y – Other specified mitochondrial myopathies
  • 8C73.Z – Mitochondrial myopathies, unspecified

Foundation children elsewhere

  • 5C53.24 – Leigh syndrome
  • 9C82.0 – Progressive external ophthalmoplegia

Sibling codes

  • 8C70 – Muscular dystrophy
  • 8C71 – Myotonic disorders
  • 8C72 – Congenital myopathies
  • 8C74 – Periodic paralyses or disorders of muscle membrane excitability
  • 8C75 – Distal myopathies
  • 8C76 – Myofibrillar myopathy
  • 8C77 – Ocular myopathy
  • 8C78 – Malignant hyperthermia or hyperpyrexia
  • 8C7Y – Other specified primary disorders of muscles
  • 8C7Z – Primary disorders of muscles, unspecified

Foundation entity

Mitochondrial myopathies – http://id.who.int/icd/entity/601991549

Part of: 08: Diseases of the nervous system