ICD-11 classes
08 Diseases of the nervous system
Diseases of neuromuscular junction or muscle
Primary disorders of muscles
8C73 — Mitochondrial myopathies

ICD-11 8C73 — Mitochondrial myopathies

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)

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 8C73 contains 6 clarifying diagnoses:

1. 8C73.0 — Autosomal recessive cardiomyopathy or ophthalmoplegia
2. 8C73.1 — Neuropathy, ataxia, and retinitis pigmentosa
3. 5C53.24 — Leigh syndrome
4. 9C82.0 — Progressive external ophthalmoplegia
5. 8C73.Y — Other specified mitochondrial myopathies
6. 8C73.Z — Mitochondrial myopathies, unspecified

The diagnosis is coded elsewhere:

• Primary disorders of muscles #10017
• Mitochondrial myopathies #10052