caa a22 4500 465768431 CHVBK 20180323111921.0 cr unu---uuuuu 170327e19900601xx s 000 0 eng 10.1007/BF00308914 doi (NATIONALLICENCE)springer-10.1007/BF00308914 Myopathy in Marinesco-Sjögren syndrome: an ultrastructural study [Elektronische Daten] [Y. Goto, A. Komiyama, Y. Tanabe, Y. Katafuchi, E. Ohtaki, I. Nonaka] Summary: Seven muscle biopsies from patients with the clinical characteristics of Marinesco-Sjögren syndrome (MSS) revealed myopathic changes of two types; muscle fiber necrosis followed by regeneration and focal myofibrillar degeneration inducing autophagocytosis with rimmed vacuole formation. In two young patients, massive muscle fiber necrosis with phagocytic invasion was the predominant feature and autophagic phenomenon was minimal, resembling the findings in progressive muscular dystrophy. Myofibrillar degeneration with autophagic phenomenon was prominent in five adult patients. The coexistence of these two degenerative processes and the secondarily induced reactive changes of muscle fiber hypertrophy, interstitial fibrosis, occasional ragged-red fibers and type 1 fiber predominance, are responsible for the wide spectrum of muscle pathology in MSS. The dense double-membrane structure surrounding myonuclei, previously reported as being specific to MSS, was present in only one biopsy. Springer-Verlag, 1990 Marinesco-Sjögren syndrome nationallicence Muscle biopsy nationallicence Electron microscopy nationallicence Autophagocytosis nationallicence Double-membrane structure nationallicence Goto Y. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut Komiyama A. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut Tanabe Y. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut Katafuchi Y. Department of Pediatrics, Kurume University School of Medicine, 830, Kurume, Japan aut Ohtaki E. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut Nonaka I. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut Acta Neuropathologica Springer-Verlag 80/2(1990-06-01), 123-128 0001-6322 80:2<123 1990 80 401 https://doi.org/10.1007/BF00308914 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00308914 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Goto Y. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut NATIONALLICENCE 700 1- Komiyama A. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut NATIONALLICENCE 700 1- Tanabe Y. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut NATIONALLICENCE 700 1- Katafuchi Y. Department of Pediatrics, Kurume University School of Medicine, 830, Kurume, Japan aut NATIONALLICENCE 700 1- Ohtaki E. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut NATIONALLICENCE 700 1- Nonaka I. Division of Ultrastructural Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (N.C.N.P.), 187, Kodaira, Tokyo, Japan aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 80/2(1990-06-01), 123-128 0001-6322 80:2<123 1990 80 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer