caa a22 4500 465768539 CHVBK 20180323111922.0 cr unu---uuuuu 170327e19900601xx s 000 0 eng 10.1007/BF00308913 doi (NATIONALLICENCE)springer-10.1007/BF00308913 Involvement of extraocular muscle in mitochondrial encephalomyopathy [Elektronische Daten] [S. Takeda, E. Ohama, F. Ikuta] Summary: We carried out a histological examination of the extraocular muscles (EOMs) in a case of myoclonus epilepsy associated with ragged-red fibers (MERRF) and two cases of mitochondrial myopathy, encephalopathy, laetic acidosis, and stroke-like episodes (MELAS), which did not manifest external ophthalmoplegia clinically. By light microscopy, many granular and vesicular fibers were seen associated with endomysial fibrosis. Electron microscopy revealed that the fibers showed prominent accumulation of abnormal mitochondria, extensive loss of myofibrils, proliferation of free sarcoplasmic reticulum and an increased amount of lipid vacuoles. These changes were more pronounced in MELAS than in MERRF. Hirano bodies were often seen in the subsarcolemmal area of muscle fibers and also in the intramuscular myelinated nerve fibers and axon terminals. These findings suggest the presence of mitochondrial myopathy of the EOMs in cases of MELAS and MERRF. Springer-Verlag, 1990 Mitochondrial encephalomyopathy nationallicence Extraocular muscle nationallicence Ultrastructure nationallicence Mitochondrial myopathy nationallicence Takeda S. Department of Pathology, Brain Research Institute, Niigata University, 951, Niigata, Japan aut Ohama E. Department of Pathology, Brain Research Institute, Niigata University, 951, Niigata, Japan aut Ikuta F. Department of Pathology, Brain Research Institute, Niigata University, 951, Niigata, Japan aut Acta Neuropathologica Springer-Verlag 80/2(1990-06-01), 118-122 0001-6322 80:2<118 1990 80 401 https://doi.org/10.1007/BF00308913 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00308913 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Takeda S. Department of Pathology, Brain Research Institute, Niigata University, 951, Niigata, Japan aut NATIONALLICENCE 700 1- Ohama E. Department of Pathology, Brain Research Institute, Niigata University, 951, Niigata, Japan aut NATIONALLICENCE 700 1- Ikuta F. Department of Pathology, Brain Research Institute, Niigata University, 951, Niigata, Japan aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 80/2(1990-06-01), 118-122 0001-6322 80:2<118 1990 80 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer