Summary
The ultrastructural examination of skeletal muscle biopsies of three typical cases of autosomal dominant inherited oculopharyngeal muscular dystrophy showed collections of tubular filaments (8.5 nm in diameter) within muscle fibre nuclei. These filaments appear to be a characteristic morphological feature of oculopharyngeal dystrophy.
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References
Barbeau, A.: The syndrome of hereditary late onset ptosis and dysphagia in French Canada. In: Progressive Muskeldystrophie, Myotonie, Myasthenie, pp. 102–109, Kuhn, E. (ed.). Berlin, Heidelberg, New York: Springer 1966
Brooke, M. H.: A clinician's view of neuromuscular diseases, pp. 123–124. Baltimore: Williams and Wilkins 1977
Fardeau, M.: Caractéristiques cytochimiques et ultrastructurales des différents types de fibres musculaires squelettiques extra-fusales (chez l'homme et quelques mammifères) Ann. Anat. Pathol. (Paris)18, 7–34 (1973)
Fardeau, M., Tomé, F. M. S.: The skeletal muscle. In: Electron microscopy in human medicine, Vol. 4, Johannessen, J. V. (ed.). New York: McGraw-Hill (in press)
Julien, J., Vital, Cl., Vallat, J. M., Le Blanc, M.: Oculopharyngeal muscular dystrophy. A case with abnormal mitochondria and “fingerprint” inclusions. J. Neurol. Sci.21, 165–169 (1974)
Man, M. X., Mikol, J., Guillard, A., Boudin, G.: Etude anatomoclinique d'une myopathie oculo-pharyngée. Bull. Soc. Ophtalmol. Fr.76, 23–30 (1976)
Mikol, J.: Pers. commun. (1979)
Neville, H. E., Brooke, M. H.: Muscle biopsy in the diagnosis of oculopharyngeal myopathy. J. Neuropathol. Exp. Neurol.33, 193, Abstract (1974)
Rebeiz, J. J., Caulfield, J. B., Adams, R. D.: Oculopharyngeal dystrophy — a presenescent myopathy. A clinico-pathologic study. In: Progress in neuro-ophthalmology. Vol. II, pp. 12–31. Brunette, J. R., Barbeau, A. (eds.), I. C. S. No. 176. Amsterdam: Excerpta Medica 1969
Sprio, A. J., Koehler, J. P., Taylor, J. M.: Oculopharyngeal dystrophy: Ultrastructural and histochemical observations of skeletal muscle. J. Neuropathol. exp. Neurol.32, 174–175 (1973)
Taylor, E. W.: Progressive vagus-glossopharyngeal paralysis with ptosis. A contribution to the group of family diseases. J. Nerv. Ment. Dis.42, 129–139 (915)
Victor, M., Hayes, R., Adams, R. D.: Oculopharyngeal muscular dystrophy. A familial disease of late life characterized by dysphagia and progressive ptosis of the eyelids. N. Engl. J. Med.267, 1267–1272 (1962)
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Tomé, F.M.S., Fardeau, M. Nuclear inclusion in oculopharyngeal dystrophy. Acta Neuropathol 49, 85–87 (1980). https://doi.org/10.1007/BF00692226
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DOI: https://doi.org/10.1007/BF00692226