Encephalomyopathies with Abnormal Mitochondria

Morphological and functional abnormalities of mitochondria have been discovered not only in various myopathies but also in multisystem disorders affecting skeletal muscle and other organs, such as the central nervous system. Primary mitochondriopathies are genuine mitochondrial diseases, generally caused by mitochondrial enzyme deficiencies. Secondary mitochondriopathies are characterized by mitochondrial reactions to extramitochondrial interference with the energy metabolism (26).