Skip to main content
Log in

Clarke's column in sporadic amyotrophic lateral sclerosis

  • Regular Papers
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Summary

Histological, ultrastructural and morphometrical observations on Clarke's column were carried out in 18 patients with sporadic amyotrophic lateral sclerosis (ALS) and 15 age-matched control subjects. Of the 18 ALS patients 6 had been on a respirator before death. Bunina bodies were found in the neuronal cytoplasm in 7 of the 12 non-respirator-supported ALS patients and in 3 of the 6 respirator-supported patients. The number of spheroids was significantly higher in the non-respirator-supported patients (P<0.01) than in the control subjects; however, the number in the respirator-supported patients was about equal to that in the controls. The number of neurons in Clarke's column in the non-respirator-supported ALS patients was not reduced, but in the respirator-supported patients they tended to disappear with time after respiratory support. These findings suggest that Clarke's column neurons are also involved primarily in the disease process in sporadic ALS. However, they may begin to disappear only after the patients require respiratory support.

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

Access this article

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

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Averback P, Crocker P (1982) Regular involvement of Clarke's nucleus in sporadic amyotrophic lateral sclerosis. Arch Neurol 39:155–156

    Google Scholar 

  2. Carpenter S (1968) Proximal axonal enlargement in motor neuron disease. Neurology 18:841–851

    Google Scholar 

  3. Chou SM (1979) Pathognomy of intraneuronal inclusion in ALS. In: Tsubaki T, Toyokura Y (eds) Amyotrophic lateral sclerosis. University of Tokyo Press, Tokyo, pp 135–176

    Google Scholar 

  4. Hayashi H, Kato S (1989) Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. J Neurol Sci 93:19–35

    Google Scholar 

  5. Hayashi H, Kato S, Kawada A, Tsubaki T (1987) Amyotrophic lateral sclerosis: oculomotor function in patients in respirator. Neurology 37:1431–1432

    Google Scholar 

  6. Hirano A (1965) Pathology of amyotrophic lateral sclerosis. In: Gajdusek DC, Gibbs CJ, Alpers M (eds) Slow, latent and temperate virus infection, NINDB Monograph No. 2. National Institutes of Health, Washington, D.C., pp 23–37

    Google Scholar 

  7. Hirano A (1973) Progress in pathology of motor neuron disease. Prog Neuropathol 2:181–215

    Google Scholar 

  8. Hirano A (1982) Aspects of the ultrastructure of amyotrophic lateral sclerosis. In: Rowland LP (ed) Human motor neuron disease. Raven Press, New York, pp 75–88

    Google Scholar 

  9. Hirano A (1986) A guide to neuropathology (in Japanese). 2nd edn. Igaku-Shoin, Tokyo, pp 344

    Google Scholar 

  10. Hirano A, Inoue K (1980) Early pathological changes of amytrophic lateral sclerosis. Electron microscopic study of chromatolysis, spheroids and Bunina bodies. Neurol Med (Tokyo) 13:148–160

    Google Scholar 

  11. 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 neuron disease. Pitman, London, pp 328–348

    Google Scholar 

  12. Holmes G (1909) The pathology of amyotrophic lateral sclerosis. Rev Neurol Psychiatry 7:693–725

    Google Scholar 

  13. Hughes JT (1982) Pathology of amyotrophic lateral sclerosis. In: Rowland LP (ed) Human motor neuron disease. Raven Press, New York, pp 61–74

    Google Scholar 

  14. Ikuta F, Makifuchi T, Ichikawa T (1979) Comparative studies of tract degeneration in ALS and other disorders. In: Tsubaki T, Toyokura Y (eds) Amyotrophic lateral sclerosis. University of Tokyo Press, Tokyo, pp 177–200

    Google Scholar 

  15. Inoue K, Hirano A (1979) Early pathological changes of amyotrophic lateral sclerosis. Autopsy findings of a case of 10 months' duration. Neurol Med (Tokyo) 11:448–455

    Google Scholar 

  16. Iwata M, Hirano A (1979) Current problems in the pathology of amyotrophic lateral sclerosis. Prog Neuropathol 4:277–298

    Google Scholar 

  17. Mulder DW (1982) Clinical limits of amytrophic lateral sclerosis. In: Rowland LP (ed) Human motor neuron diseases. Raven Press, New York, pp 15–22

    Google Scholar 

  18. Okamoto K, Hirano A (1982) Bunina bodies in amyotrophic lateral sclerosis. Neurol Med (Tokyo) 17:259–265

    Google Scholar 

  19. Okamoto K, Yamazaki T, Yamaguchi H, Shooji M, Hirai S (1988) Pathology of Clarke's nucleus in sporadic amyotrophic lateral sclerosis. Clin Neurol (Tokyo) 28:536–542

    Google Scholar 

  20. Oyanagi K, Makifuchi T, Ikuta F (1983) A topographic and quantitative study of neurons in human spinal gray matter, with special reference to their changes in amyotrophic lateral sclerosis. Biomed Res 4:211–224

    Google Scholar 

  21. Oyanagi K, Ikuta F, Horikawa Y (1989) Evidence for sequenctial degeneration of the neurons in the intermediate zone of the spinal cord in amyotrophic lateral sclerosis: a topographic and quantitative investigation. Acta Neuropathol 77:343–349

    Google Scholar 

  22. Rossi GL, Luginbühl H, Probst D (1970) A method for ultrastructural study of lesions found in conventional histological sections. Virchows Arch [A] 350:216–224

    Google Scholar 

  23. Rowland LP (1982) Diverse forms of motor neuron disease. In: Rowland LP (ed) Human motor neuron disease. Raven Press, New York, pp 1–13

    Google Scholar 

  24. Sasaki S, Kamei H, Yamane K, Murayama S (1988) Swelling of neuronal processes in motor neuron disease. Neurology 38:1114–1118

    Google Scholar 

  25. Sasaki S, Murayama S, Yamane K, Sakuma H, Takeishi M (1989) Swelling of proximal axons in a case of motor neuron disease. Ann Neurol 25:520–522

    Google Scholar 

  26. Smith MC (1960) Nerve fiber degeneration in the brain in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 23:269–282

    Google Scholar 

  27. Takahashi H, Ohama E, Ikuta F (1991) Are Bunina bodies of endoplasmic reticulum origin? An ultrastructural study of subthalamic eosinophilic inclusions in a case of atypical motor neuron disease. Acta Pathol Jpn 41:889–894

    Google Scholar 

  28. Takahashi H, Ohama E, Ikuta F (1991) Motor neuron disease: two forms of Bunina body revealed by a method for ultrastructural study of conventional histological sections. In: Ikuta F (ed) Neuropathology in Brain Research. Elsevier, Amsterdam, pp 127–132

    Google Scholar 

  29. Tandan R, Bradley WG (1985) Amyotrophic lateral sclerosis. Part 1: Clinical features, pathology, and ethical issues in management. Ann Neurol 18:271–280

    Google Scholar 

  30. Tomonaga M, Saito M, Yoshimura M, Shimada H, Tohgi H, Yamanouchi H, Mochizuki H (1978) On the intracytoplasmic inclusions (Bunina bodies) observed in the nerve cells of amyotrophic lateral sclerosis. Shinkei Kenkyu no Shinpo 22:497–510

    Google Scholar 

  31. Tomonaga M, Saito M, Yoshimura M, Shimada H, Tohgi H (1978) Ultrastructure of the Bunina bodies in anterior horn cells of amyotrophic lateral sclerosis. Acta Neuropathol (Berl) 42:81–86

    Google Scholar 

  32. Williams C, Kozlowski MA, Hinton DR, Miller CA (1990) Degeneration of spinocerebellar neurons in amyotrophic lateral sclerosis. Ann Neurol 27:215–225

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

Supported in part by a research grant for CNS degenerative diseases from the Ministry of Health and Welfare, Japan

Rights and permissions

Reprints and permissions

About this article

Cite this article

Takahashi, H., Oyanagi, K., Ohama, E. et al. Clarke's column in sporadic amyotrophic lateral sclerosis. Acta Neuropathol 84, 465–470 (1992). https://doi.org/10.1007/BF00304464

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00304464

Key words

Navigation