Acta Neuropathologica

, Volume 75, Issue 5, pp 523–528 | Cite as

Ultrastructural study of chromatolytic neurons in an adult-onset sporadic case of amyotrophic lateral sclerosis

  • H. Kusaka
  • T. Imai
  • S. Hashimoto
  • T. Yamamoto
  • K. Maya
  • M. Yamasaki
Case Reports

Summary

Ultrastructural features of chromatolytic neurons observed in a sporadic case with amyotrophic lateral sclerosis (ALS) are reported. A 70-year-old woman died of weakness and atrophy of the four limbs, bulbar and facial muscles, and hyperreflexia, of 3 1/2 years' duration. Neuronal loss was marked in the anterior horn of the spinal cord, with degeneration of the pyramidal tracts. Most of the remaining neurons showed chromatolysis. Some of the chromatolytic neurons contained faintly eosinophilic inclusions with a halo. Few spheroids were observed. Hypoglossal nuclei, nucleus ambiguus, motor nuclei of N. VII and N.V were well populated, but contained several chromatolytic neurons. Ultrastructurally, the chromatolytic neurons contained aggregates of fibrils thicker than the 10-nm neurofilaments. These fibrils were arranged randomly, and were closely associated with granular materials as well as rough endoplasmic reticulum. Neurofilamentous accumulations reported to be common in sporadic ALS were rare in this case. No Bunina body was observed.

Key words

Amyotrophic lateral sclerosis Chromatolysis Ultrastructure Motor neuron disease Hyaline intraneuronal inclusions 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Delisle MB, Carpenter S (1984) Neurofibrillary axonal swellings and amyotrophic lateral sclerosis. J Neurol Sci 63:241–250Google Scholar
  2. 2.
    Hirano A (1965) Pathology of amyotrophic lateral sclerosis. In: Gajdusek DC, Gibbs CJ Jr (eds) NINDB monograph, no 2, Slow, latent and temperate virus infections. National Institute of Health, Washington, pp 23–27Google Scholar
  3. 3.
    Hirano A (1981) A guide to neuropathology. Igaku-shoin, Tokyo, p 110Google Scholar
  4. 4.
    Hirano A (1982) Aspects of the ultrastructure of amyotrophic lateral sclerosis. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 75–88Google Scholar
  5. 5.
    Hirano A, Iwata M (1978) Pathology of motor neurons with special reference to amyotrophic lateral sclerosis and related diseases. In: Tsubaki T, Toyokura Y (eds) Amyotrophic lateral sclerosis. University of Tokyo, Tokyo, pp 107–133Google Scholar
  6. 6.
    Hirano A, Donnenfeld H, Sasaki S, Nakano I, Bartfeld H (1984) The fine structure of motor neuron disease. In: Rose FC (ed) Research progress in motor neurone disease. Pitman, London, pp 328–345Google Scholar
  7. 7.
    Hirano A, Donnenfeld H, Sasaki S, Nakano I (1984) Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 43:461–470Google Scholar
  8. 8.
    Hirano A, Nakano I, Kurland LT, Mulder DW, Holley PW, Saccomanno G (1984) Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 43:471–480Google Scholar
  9. 9.
    Iwata M, Hirano A (1979) Current problems in the pathology of amyotrophic lateral sclerosis. Prog Neuropathol 4:277–298Google Scholar
  10. 10.
    Kuroda S, Kuyama K, Morioka E, Ohtsuki S (1986) Sporadic amyotrophic lateral sclerosis with intracytoplasmic eosinophilic inclusions. A case closely akin to familial ALS. Neurol Med (Tokyo) 24:31–37Google Scholar
  11. 11.
    Kusaka H, Hirano A (1985) Fine structure of anterior horns in patients without amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 44:430–438Google Scholar
  12. 12.
    Kusaka H, Donnenfeld H, Hirano A (1985) Bundles of ribosome-associated linear structures. Neurol Med (Tokyo) 23:96–98Google Scholar
  13. 13.
    Mizusawa H, Hirano A (1987) Lower motor neurons disease associated with a focal onion bulb formation in an anterior root. Neurol Med (Tokyo) 26:309–311Google Scholar
  14. 14.
    Nakano I, Donnenfeld H, Hirano A (1983) A neuropathological study of amyotrophic lateral sclerosis. With special reference to central chromatolysis and spheroid in the spinal ventral horn and some pathological changes of the motor cortex. Neurol Med (Tokyo) 18:136–144Google Scholar
  15. 15.
    Nakano I, Hirano A, Kurland LT, Mulder DW, Holley PW, Saccomanno G (1984) Familial amyotrophic lateral sclerosis. Neuropathology of two brothers in American “C” family. Neurol Med (Tokyo) 20:458–471Google Scholar
  16. 16.
    Oda M, Akagawa N, Tabuchi Y, Tanabe H (1978) A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions. Acta Neuropathol (Berl) 44:211–216Google Scholar
  17. 17.
    Takahashi K, Nakamura H, Okada E (1972) Hereditary amyotrophic lateral sclerosis. Histochemical and electron microscopic study of hyaline inclusions in motor neurons. Arch Neurol 27:292–299Google Scholar

Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • H. Kusaka
    • 1
  • T. Imai
    • 1
  • S. Hashimoto
    • 2
  • T. Yamamoto
    • 1
  • K. Maya
    • 1
  • M. Yamasaki
    • 1
  1. 1.Department of Neurology, Neurological CenterKitano HospitalKita-ku, OsakaJapan
  2. 2.Department of NeurologyTenri HospitalTenri city, NaraJapan

Personalised recommendations