Summary
Muscle biopsies from 5 cases of Werdnig-Hoffmann disease and 4 cases of ALS were investigated by histology and electronmicroscopy. The differences in morphology found in the atrophic muscle fibres in ALS and in W.H. diesease consisted mainly in the shape and structure of their cells and the number of the nuclei.
Atrophic muscle fibres irregular in shape and size with degenerative changes and accumulated nuclei observed in ALS were markedly different from the picture of the fibres in W.H. disease. The presence of small muscle cells uniform diameter with well preserved architecture and single nuclei and numerous myotube-like cells seem to indicate a foetal defect in children with W.H. disease.
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Afifi, K. A., Aleu, F. P., Godgeld J., MacKay, B.: Ultrastructure of atrophic muscle in amyotrophic lateral sclerosis. Neurology (Minneap.)5, 475–481 (1966)
Fidziańska, A.: Ultrastructural changes in muscle in spinal muscular atrophy Werdnig-Hoffmann's. Acta neuropath. (Berl.)27, 247–256 (1974)
Fidziańska, A.: Electron microscopic study of the development of human foetal muscle. In: Structure and function of normal and diseased muscle and peripheral nerve (eds. I. Hausmanowa-Petrusewicz, A. Jędrzejowska), pp. 15–20. Polish Medical Publisher 1974
Fischmann, D. A.: The fine structure of muscle differentation in monolayer culture. In: Regeneration of striated muscle and myogenesis (ed. A. Mauro), pp. 88–104. Amsterdam: Excerpta Medica 1972
Gauthier, G. F., Dunn, R. A.: Ultrastructural and cytochemical feature of mammalian skeletal muscle fibres following denervation. J. Cell. Sci.12, 525–547 (1973)
Von Haelst, V.: An electron microscopic study of muscle in Werdnig-Hoffmann's disease. Virchows Arch., Abt. A, Path. Anat.351, 291–305 (1970)
Hausmanowa-Petrusewicz, I., et al.: Infantile and juvenile spinal muscular atrophy. J. neurol. Sci.6, 269–287 (1968)
Hughes, J. T., Brownell, B.: Ultrastructure of muscle in Werdnig-Hoffmann disease. J. neurol. Sci.8, 363–379 (1969)
Ishikawa, H.: Satellite cells in developing muscle and tissue culture. In: Regeneration of striated muscle and myogenesis (ed. A. Mauro), pp. 167–179. Amsterdam: Excerpta Medica 1970
Larson, P. F., Jenkinson, M., Hudgson, P.: The morphological development of chick embryo skeletal muscle grown in tissue culture as studied by electron microscopy. J. neurol. Sci.10, 384–405 (1970)
Lee, J. C., Altschul, R.: Electron microscopy of the nuclei of denervated skeletal muscle. Z. Zellforsch.61, 168–182 (1963)
Mastaglia, F. L., Walton, J. N.: An electron microscopic study of skeletal muscle from case of the Kugelberg-Welander syndrome. Acta neuropath. (Berl.)17, 201–219 (1971)
Miledi, R., Slater, C. R.: Some mitochondrial changes in denervated muscle. J. Cell. Sci.3, 49–54 (1968)
Muscatello, U., Patriarca, P. L.: Denervation and disuse atrophy in pigeon breast muscle an electron microscopic study. Amer. J. Path.52, 1169–1189 (1968)
Nakashima, N.: Inclusion bodies in denervated skeletal muscle of mice. Nagoya J. med. Sci.33, 189–202 (1970)
Ochoa, J., Mair, W. G. P.: The ultrastructure of normal foetal muscle and foetal muscle of known dystrophic carriers. The Proceedings of the Fourthy Symposium on Current Research in Dystrophy held at the National Hospital, Queen Square London W.C.I., 11th–12th January, 1968
Rebeiz, J. J.: Endomysial cell proliferation and sarcolemmal nuclear condensation in denervated muscles: an autoradiographic study. Clinical studies in myology. Proc. 2nd Int. Congr. Musc. Dis. (ed. B. A. Kalkulas), pp. 668–674. Amsterdam: Excerpta Medica 1973
Recondo, J., Fardeau, M., Lapresle, J.: Étude au microscope électronique des lésions musculaires d'atrophie neurogene par atteinte de la corne anterieure. Rev. neurol.114, 169–192 (1966)
Roth, R. G., Graziani, L. J., Terry, R. D., Scheinberg, L. O.: Muscle fine structure in the Kugelberg-Welander syndrome chronic spinal muscular atrophy. J. Neuropath. exp. Neurol.24, 444–454 (1965)
Roy, S., Dubowitz, V., Volman, L.: Ultrastructure of muscle in infantile spinal muscular atrophy. J. neurol. Sci.12, 219–232 (1971)
Sato, T., Tsubaki, T.: Electron microscopic studies on the muscle of neuromuscular disease. Clin. Neurol. (Tokyo)8, 3–12 (1968)
Schaffino, S., Settembrini, P.: Studies on the effect of denervation in developing muscle. I. Differentiation of sarcotubular system. Virchows. Arch., Abt. B, Zellpath.4, 345–356 (1970)
Schimada, Y.: Electron microscope observation on the fusion of chick myoblast in vitro. J. Cell Biol.48, 128–142 (1971)
Shafiq, S. A., Milhorat, A. T., Gorycki, M. A.: Fine structure of human muscle in neurogenic atrophy. Neurology (Minneap.)17, 934–948 (1967)
Stronnington, H. H., Engel, A. G.: Normal and denervated muscle. A morphometric study of fine structure. Neurology (Minneap.)23, 714–724 (1973)
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This work was supported by the Polish Academy of Science within the project 09.4.1.
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Fidziańska, A. Morphological differences between the atrophied small muscle fibres in amyotrophic lateral sclerosis and Werdnig-Hoffmann disease. Acta Neuropathol 34, 321–327 (1976). https://doi.org/10.1007/BF00696561
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DOI: https://doi.org/10.1007/BF00696561