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Adult-onset chronic progressive external ophthalmoplegia with mitochondrial myopathy

ORPHA:329336· ICD-10 G71.3

Definition

A rare mitochondrial disease characterized by adult onset of progressive external ophthalmoplegia, exercise intolerance, muscle weakness, manifestations of spinocerebellar ataxia (e.g. impaired gait, dysarthria) and mild motor peripheral neuropathy. Respiratory insufficiency has been reported in some cases.

Prevalence
Unknown
Inheritance
Autosomal dominant, Mitochondrial inheritance
Age of onset
Adult