Acta Neuropathologica

, Volume 80, Issue 6, pp 611–617 | Cite as

Chronic progressive and relapsing neuromyopathy with massive dilatations of endoplasmic reticulum in muscle fibers

  • B. Lach
  • S. Christie
  • D. Preston
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Summary

Large intracytoplasmic inclusions arising from the endoplasmic reticulum and nuclear envelope were found in the muscle fibers of a 32-year-old individual with a life-long, chronic and progressive sensory-motor neuropathy. The morphological features of the inclusions were similar to that occasionally reported in the striated muscles in several unrelated conditions as well as to “hyaline” or “colloid” inclusions commonly seen in motor neurons of the brain stem and spinal cord. The chemical nature of the inclusions is not known. Their occurrence in the muscle fibers is probably secondary to chronic denervation.

Key words

Intranuclear inclusions Endoplasmic reticulum Hyaline inclusions Striated muscle Ultrastructure 
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References

  1. 1.
    Afifi AK, Aleu FP, Goodgold J, Mackay B (1966) Ultrastructure of atrophic muscle in amyotrophic lateral sclerosis. Neurology 16:475–481Google Scholar
  2. 2.
    Blom J, Mansa B, Wiik A (1976) A study of Russell bodies in human monoclonal plasma cells by means of immunofluorescence and electron microscopy. Acta Pathol Microbiol Scand [A] 84:35–349Google Scholar
  3. 3.
    Brooke MH (1986) Clinical review of neuromuscular diseases. Williams and Wilkins, BaltimoreGoogle Scholar
  4. 4.
    Carpenter S, Karpati G (1984) Pathology of skeletal muscle. Churchill Livingstone, New York, p 292Google Scholar
  5. 5.
    Carpenter S, Carpati G, Rothman S, Watters G, Andermann F (1978) Pathological involvement of primary sensory neurons in Werdnig-Hoffmann disease. Acta Neuropathol (Berl) 42: 91–97Google Scholar
  6. 6.
    Culebras A, Feldman RG, Merk FB (1973) Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy. J Neurol Sci 19:319–329Google Scholar
  7. 7.
    Dubowitz V (1978) Muscle disorders in childhood. In: Schaffer AJ, Markowitz M (eds) Major problems in clinical pediatrics, vol 16. W. B. Saunders, London, pp 19–255Google Scholar
  8. 8.
    Dubowitz V, Brooke MH, Neville HE (1973) Muscle biopsy: a modern approach. In: Waffan JN (ed) Major problems in neurology. W. B. Saunders, London Philadelphia Toronto, pp 20–33Google Scholar
  9. 9.
    Duchen LW (1973) The local effects of tetanus toxin on the electron microscopic structure of skeletal muscle fibres of the mouse. J Neurol Sci 19:169–177Google Scholar
  10. 10.
    Dyck PJ, Thomas PK; Lambert EH, Bunge R (1984) Peripheral neuropathy, vol 1 and 2, 2nd edn. W. B. Saunders, LondonGoogle Scholar
  11. 11.
    Engel AG (1970) Evolution and content of vacuoles in primary hypokalemic periodic paralysis. Mayo Clin Proc 4:774–814Google Scholar
  12. 12.
    Engel AG, Banker BQ (1986) Ultrastructural changes in the diseased muscle. In: Engel AG, Banker BQ (eds) Myology. McGraw-Hill, New York, pp 909–1043Google Scholar
  13. 13.
    Ghadially FN (1988) Ultrastructural pathology of the cell and matrix, vol 1 3rd edn. Butterworths, Toronto, pp 78–80, 413–550Google Scholar
  14. 14.
    Goulon M, Fardeau M, Got Cl, Babinet P, Manko E (1976) Myopathie centro-nucleaire d'expression clinique tardive. Rev Neurol (Paris) 4:275–290Google Scholar
  15. 15.
    Hausmanowa-Petrusewicz I, Askanas W, Badurska B, Emeryk B, Fidzianska A, Garbalinska W, Hetnarska L, Jedrzejowska H, Kamieniecka Z, Niebroj-Dobosz I, Prot J, Sawicka E (1968) Infantile and juvenile spinal muscular atrophy. J Neurol Sci 6:269–287Google Scholar
  16. 16.
    Kawabuchi M (1982) Neostigmine myopathy is a calcium ion-mediated myopathy initially affecting the motor endplate. J Neuropathol Exp Neurol 41:298–314Google Scholar
  17. 17.
    Lach B, Russell N, Benoit B, Atack D (1988) Cellular blue nevus (“melanocytoma”) of the spinal meninges: electron microscopic and immunohistochemical features. Neurosurgery 22:773–780Google Scholar
  18. 18.
    Marshall A, Duchin IW (1975) Sensory system involvement in infantile spinal muscular atrophy. J Neurol Sci 26:349–359Google Scholar
  19. 19.
    Mastaglia FL, Hudgson P (1981) Ultrastructural studies of diseased muscle. In: Walton J (ed) Disorders of the voluntary muscle. Churchill Livingstone, London, pp 296–356Google Scholar
  20. 20.
    Mendell JR, Markesbery WR (1971) Neuronal intracytoplasmic hyaline inclusions. J Neuropathol Exp Neurol 30:233–239Google Scholar
  21. 21.
    Namba T, Nakamura T, Grob D (1967) Staining for nerve fiber and cholinesterase activity in fresh frozen sections. Am J Clin Pathol 47:74–77Google Scholar
  22. 22.
    Norman MG (1974) Hyaline (“colloid”) cytoplasmic inclusions in motoneurons in association with familial micrencephaly, retardation and seizures. J Neurol Sci 23:63–70Google Scholar
  23. 23.
    Ouvrier RA, McLeod JG, Morgan GJ, Wise GA, Conchin TE (1981) Hereditary motor and sensory neuropathy of neuronal type with onset in erly childhood. J Neurol Sci 51:181–197Google Scholar
  24. 24.
    Pelligrino C, Franzini C (1963) An electron microscope study of denervation atrophy in red and white skeletal muscle fibers. J Cell Biol 17:327–349Google Scholar
  25. 25.
    Roessman U, MacFarland DE (1971) Hyaline intracytoplasmic inclusions in motor neurons. J Neuropathol Exp Neurol 30:551–556Google Scholar
  26. 26.
    Roth RG, Graziani LJ, Terry RD, Scheinberg LC (1965) Muscle fine structured in Kugelberg-Welander Syndrome (chronic spinal muscular atrophy). J Neuropathol Exp Neurol 24:444–454Google Scholar
  27. 27.
    Saghal V, Saghal S (1977) A new congenital myopathy. Acta Neuropathol (Berl) 37:225–230Google Scholar
  28. 28.
    Schroder JM, Adams RD (1968) The ultrastructural morphology of the muscle fiber in myotonic dystrophy. Acta Neuropathol (Berl) 10:218–241Google Scholar
  29. 29.
    Serratrice G, Roux H (1979) Peroneal atrophies and related disorders. Masson, ParisGoogle Scholar
  30. 30.
    Shafiq SA, Milhorat AT, Gorycki MA (1967) Fine structure of human muscle in neurogenic atrophy. J Neurol Sci 8:363–379Google Scholar
  31. 31.
    Sternberger LA (1979) Immunocytochemistry, 2nd edn. John Wiley & Sons, New York, pp 104–170Google Scholar
  32. 32.
    Szliwowski HB, Drochmans P (1975) Ultrastructural aspects of muscle and nerve in Werdnig-Hoffman disease. Acta Neuropathol (Berl) 31:281–296Google Scholar
  33. 33.
    Takei Y, Samuels-Mirra S (1971) Intracytoplasmic hyaline inclusion bodies in the nerve cells of the hypoglossal nucleus in human autopsy material. Acta Neuropathol (Berl) 17:14–23Google Scholar
  34. 34.
    Trevor-Hughes JH, Brownelle B (1969) Ultrastructure of muscle in Werdnig-Hoffman disease. J Neurol Sci 8:363–379Google Scholar
  35. 35.
    Turel AP, Levinsohn MW, Derakhshan I, Gutierrez Y (1975) Reye syndrome and cerebellar intracytoplasmic inclusion bodies. Arch Neurol 32:624–628Google Scholar
  36. 36.
    Vital A, Vital C, Coquette M (1986) Chronic peripheral neuropathy: unusual nerve and muscle biopsy findings. Muscle Nerve 9:471–472Google Scholar
  37. 37.
    Wisotzkey HM, Moossy J (1972) Hyaline (“colloid”) cytoplasmic inclusions in neurons of human hypoglossal nuclei. Arch Pathol 93:61–70Google Scholar

Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • B. Lach
    • 1
  • S. Christie
    • 2
  • D. Preston
    • 2
  1. 1.Department of Laboratory Medicine, Ottawa Civic HospitalUniversity of OttawaOttawaCanada
  2. 2.Division of Neurology, Ottawa Civic HospitalUniversity of OttawaOttawaCanada

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