Multiple mtDNA deletions have been reported to be a cause of inherited
recurrent myoglobinuria. We report a 57-year-old man with autosomal d
ominant progressive external ophthalmoplegia and multiple mtDNA deleti
ons who developed acute rhabdomyolysis provoked by alcohol. A repeated
alcohol intake resulted in a 57-fold increase in serum myoglobin. Pat
ients with mitochondrial myopathy and multiple mtDNA deletions, regard
less of associated phenotype and mode of inheritance, may develop rhab
domyolysis.