Skip to main content
SpringerLink
Log in

Oculopharyngeal dystrophy: Ultrastructure of muscles distant from the primary myopathy

  • Original Works
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

The pectoral and psoas muscles from a 72-year-old man afflicted with oculopharyngeal muscular dystrophy were processed at autopsy for electron microscopy. The ultrastructural analyses of the pectoral muscle showed myofibrils which exhibited Z line streaming and a general breakdown in the organization of the sarcomere. In addition, some of the myofibrils displayed sites of degeneration at the center of the A band. The changes in the psoas muscles which are distant from the primary myopathic loci of oculopharyngeal muscular dystrophy were more extensive than that seen in the pectoral muscle. Control tissues showed intact myofibrils and little postmortem alteration.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Aarli JA (1969) Oculopharyngeal muscular dystrophy. Acta Neurol Scand 45:484–492

    Google Scholar 

  • Barbeau A (1966) The syndrome of hereditary late-onset ptosis and dysphagia in French Canada. In: Kuhn E (ed) Progressive muscular dystrophy, myotonia, myasthenia. Springer, Berlin Heidelberg New York, pp 102–109

    Google Scholar 

  • Bosch EP, Gowans JDC, Munsat T (1979) Inflammatory myopathy in oculopharyngeal dystrophy. Muscle Nerve 2:73–77

    Google Scholar 

  • Brooke MH (1977) A clinician's view of neuromuscular diseases. Williams and Wilkins, Baltimore, pp 123–124

    Google Scholar 

  • Collan Y, Salmenperä M (1976) Electron microscopy of post mortem autolysis of rat muscle tissue. Acta Neuropathol (Berl) 35:219–233

    Google Scholar 

  • Dubowitz V, Brooke MH (1973) Muscle biopsy: A modern approach. Saunders, London, pp 231–241

    Google Scholar 

  • Duranceau A, Forand MD, Fauteux JP (1980) Surgery in oculopharyngeal muscular dystrophy. Am J Surg 139:33–39

    Google Scholar 

  • Fried K, Arlozorov A, Spira R (1975) Autosomal recessive oculopharyngeal muscular dystrophy. J Med Genet 12:416–418

    Google Scholar 

  • Johnson CC, Kuwabara T (1974) Oculopharyngeal muscular dystrophy. Am J Ophthalmol 77:872–879

    Google Scholar 

  • Julien J, Vital Cl, Vallat JM, Vallat M, LeBlanc M (1974) Oculopharyngeal muscular dystrophy. A case with abnormal mitochondria and “fingerprint” inclusions. J Neurol Sci 21:165–169

    Google Scholar 

  • Kozachek JW, Irish MJ, Illovsky P, Wilson FJ (1980) Degeneration of muscle distant from the primary myopathy in oculopharyngeal dystrophy. Anat Rec 196:103A

    Google Scholar 

  • Lewis I (1969) Oculopharyngeal muscular dystrophy. A family study. In: Brunette J-R, Barbeau A (eds) Progress in neuro-ophthalmology, vol 2. Proc 2nd Int Congr Neurogenet Neuroophthalmol World Fed Neurol. Excerpta Medica, Amsterdam, pp 4–11

    Google Scholar 

  • Mair WGP, Tomé FMS (1972) Atlas of the ultrastructure of diseased human muscle. Churchill Livingstone, Edinburgh, pp 46–137

    Google Scholar 

  • Murphy SF, Drachman DB (1968) The oculopharyngeal syndrome. JAMA 203:1003–1008

    Google Scholar 

  • Rebeiz JJ, Caulfield JB, Adams RD (1969) Oculopharyngeal dystrophy — a presenescent myopathy. A clinico-pathologic study. In: Brunette J-R, Barbeau A (eds) Progress in neuro-ophthalmology, vol 2. Proc 2nd Int Congr Neurogenet Neuro-ophthalmol World Fed Neurol. Excerpta Medica, Amsterdam, pp 12–31

    Google Scholar 

  • Shy GM, Silverstein I (1965) A study of the effects upon the motor unit by remote malignancy. Brain 88:515–528

    Google Scholar 

  • Silverman H, Hikida RS, Staron RS (1979) Loss of thick filaments from fast-twitch glycolytic muscle fibers of the pigeon pectoralis after chronic administration of dantrolene sodium. Am J Anat 155:69–82

    Google Scholar 

  • Takahashi K, Nakamura F, Inoue A (1981) Postmortem changes in the actin-myosin interaction of rabbit skeletal muscle. J Biochem 89:321–324

    Google Scholar 

  • Taylor EW (1915) Progressive vagus-glossopharyngeal paralysis with ptosis. A contribution to the group of family diseases. J Nerv Ment Dis 42:129–139

    Google Scholar 

  • Tomé FMS, Fardeau M (1980) Nuclear inclusions in oculopharyngeal dystrophy. Acta Neuropathol (Berl) 49:85–87

    Google Scholar 

  • Victor M, Hayes R, Adams RD (1962) 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

    Google Scholar 

  • Weitzner S (1971) The histopathology of the pharynx and esophagus in oculopharyngeal muscular dystrophy. Am J Gastroenterol 56:378–382

    Google Scholar 

  • Yarom R, Shapira Y (1977) Myosin degeneration in a congenital myopathy. Arch Neurol 34:114–115

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Dept. of Anatomy, Rutgers Medical School, College of Medicine and Dentistry of New Jersey, 08854, Piscataway, NJ, USA

    J. W. Kozachek & F. J. Wilson

Authors
  1. J. W. Kozachek
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. F. J. Wilson
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kozachek, J.W., Wilson, F.J. Oculopharyngeal dystrophy: Ultrastructure of muscles distant from the primary myopathy. Acta Neuropathol 57, 7–12 (1982). https://doi.org/10.1007/BF00688872

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00688872

Key words

Search

Navigation