Mitochondrial myopathies

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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)

code elsewhere

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

sections/codes in this section (8C73-8C73)

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

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