ICD-11 code : 8C73.0;
Preferred Label : Autosomal recessive cardiomyopathy or ophthalmoplegia;
ICD-11 definition : Autosomal recessive cardiomyopathy and ophthalmoplegia is a childhood-onset disease
characterized by progressive external ophthalmoplegia, mild facial and proximal limb
weakness, and severe cardiomyopathy. Muscle biopsies shows ragged-red and cytochrome
C oxidase-negative fibers; the activities of several complexes in the electron-transport
chain are decreased. The combination of progressive external ophthalmoplegia, cardiomyopathy,
and multiple mtDNA deletions is thought to be due to a defect of communication between
the nuclear and mitochondrial genomes.;
ICD-11 synonym : Autosomal recessive cardiomyopathy and ophthalmoplegia; ARCO - [Autosomal recessive cardiomyopathy and ophthalmoplegia];
ICD-11 acronym : ARCO;
Origin ID : 607844076;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
Autosomal recessive cardiomyopathy and ophthalmoplegia is a childhood-onset disease
characterized by progressive external ophthalmoplegia, mild facial and proximal limb
weakness, and severe cardiomyopathy. Muscle biopsies shows ragged-red and cytochrome
C oxidase-negative fibers; the activities of several complexes in the electron-transport
chain are decreased. The combination of progressive external ophthalmoplegia, cardiomyopathy,
and multiple mtDNA deletions is thought to be due to a defect of communication between
the nuclear and mitochondrial genomes.
