Elektronenmikroskopische Befunde bei 3 Fallen von chronisch progressiver okulärer Muskeldystrophie

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Abstract
Muscle biopsies, taken from patients showing clinical symptoms of progressive ocular muscle dystrophy, were examined microscopically, histochemically and under the electronic microscope. The isolated dystrophic lesions were characterized by swelling of mitochondria and of the sarcoplasmatic reticulum and by the disintegration of the striation. In addition, in some extra-ocular and shoulder muscles enlarged mitochondria were seen with their inner membrane arranged parallel or concentrically. These were also found together with giant sarcosomes containing granules in paracrystalline form in their enlarged lumen. These together form crystalloids. Such giant mitochondria can disintegrate, when the liberated crystalloids surrounded by a membrane lie freely in the cytoplasm.