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Sporadic motor neuron disease with Lewy body-like hyaline inclusions

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Summary

Lewy body-like hyaline inclusions were immunocytochemically and electron microscopically investigated in a patient with sporadic motor neuron disease. The hyaline inclusions were chiefly observed within the perikarya of both normal-looking and chromatolytic anterior horn cells in the lumbar spinal cord, but some were detected in the axons and dendrites. Usually, a single inclusion was found in the perikaryon, but in rare cases two or more were observed. Immunocytochemically, these inclusions were intensely immunostained with anti-ubiquitin anti-body. Ultrastructurally, the hyaline inclusions were chiefly composed of randomly arranged linear structures associated with ribosome-like granules, varying from compactly arranged linear densities to more loosely packed ones. They contained scattered vesicles of various sizes and occasionally a focal accumulation of randomly arranged 10-nm neurofilaments or 13–25-nm filamentous structures.

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References

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

    Google Scholar 

  2. Delisle MB, Carpenter S (1984) Neurofibrillary axonal swellings and amyotrophic lateral sclerosis. J Neurol Sci 63:241–250

    Google Scholar 

  3. Den Hartog Jager WA, Bethlem J (1960) The distribution of Lewy bodies in the central and autonomic nervous systems in idiopathic paralysis agitans. J Neurol Neurosurg Psychiatry 23:283–290

    Google Scholar 

  4. Dickson DW, Yen S-H, Suzuki KI, Davies P, Garcia JH, Hirano A (1986) Ballooned neurons in select neuro-degenerative disease contain phosphorylated neurofilament epitopes. Acta Neuropathol (Berl) 71:216–223

    Google Scholar 

  5. Forno LS, Langston JW, DeLanney LE, Irwin I (1988) An electron microscopic study of MPTP-induced inclusion bodies in an old monkey. Brain Res 448:150–157

    Google Scholar 

  6. Hirano A, Kurland LT, Sayre GP (1967) Familial amyotrophic lateral sclerosis. A subgroup characterized by posterior and spinocerebellar tract involvement and hyaline inclusions in the anterior horn cells. Arch Neurol 16:232–243

    Google Scholar 

  7. Hirano A, Donnenfeld H, Sasaki S, Nakano I (1984) Fine structural observations of neurofilamentous changes in amytrophic lateral sclerosis. J Neuropathol Exp Neurol 43:461–470

    Google Scholar 

  8. Hirano A, Nakano I, Kurland CT, Mulder DW, Holley PW, Saccomanno G (1984) Fine structural study of neurofibrillary changes in a family with amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 43:471–480

    Google Scholar 

  9. Hughes JT, Jerrome D (1971) Ultrastructure of anterior horn motor neurons in the Hirano-Kurland-Sayre type of combined neurological system degeneration. J Neurol Sci 13:389–399

    Google Scholar 

  10. Ihara Y, Nukina N, Miura R, Ogawara M (1987) Phosphorylated tau protein is integrated into paired helical filaments in Alzheimer's disease. J Biochem 99:1807–1811

    Google Scholar 

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

    Google Scholar 

  12. Kato T, Katagiri T, Hirano A, Sasaki H, Arai S (1988) Sporadic lower motor neuron disease with Lewy body-like inclusions: a new subgroup? Acta Neuropathol 76:208–211

    Google Scholar 

  13. Kosaka K (1978) Lewy bodies in cerebral cortex. Report of three cases. Acta Neuropathol (Berl) 42:127–134

    Google Scholar 

  14. Kosaka K, Yoshimura M, Ikeda K, Budka H (1984) Diffuse type of Lewy body disease: a progressive dementia with abundant cortical lewy bodies and senile changes of varying degree — A new disease? Clin Neuropathol 3:185–192

    Google Scholar 

  15. Kuroda S, Kuyama K, Morioka E, Ohtsuki S, Nanba R (1986) Sporadic amyotrophic lateral sclerosis with intracytoplasmic eosinophilic inclusions. A case closely akin to familial ALS. Neurol Chir (Tokyo) 24:31–37

    Google Scholar 

  16. Kusaka H, Imai T, Hashimoto S, Yamamoto T, Maya K, Yamasaki M (1988) Ultrastructural study of chromatolytic neurons in an adult-onset sporadic case of amyotrophic lateral sclerosis. Acta Neuropathol (Berl) 75:523–528

    Google Scholar 

  17. Leigh PN, Anderton BH, Dodson A, Gallo J-M, Swash M, Power DM (1988) Ubiquitin deposits in anterior horn cells in motor neuron disease. Neurosci Lett 93:197–203

    Google Scholar 

  18. Lowe J, Lennox G, Jefferson D, Morrell K, McQuire D, Gray T, Landon M, Doherty FJ, Mayer RJ (1988) A filamentous inclusion body within anterior horn neurons in motor neuron disease defined by immunocytochemical localization of ubiquitin. Neurosci Lett 94:203–210

    Google Scholar 

  19. Mori H, Kondo J, Ihara Y (1987) Ubiquitin is a component of paired helical filaments in Alzheimer's disease. Science 235:1641–1644

    Google Scholar 

  20. Munoz DG, Greene C, Perl DP, Selkoe DJ (1988) Accumulation of phosphorylated neurofilaments in anterior horn motoneurons of amyotrophic lateral sclerosis patients. J Neuropathol Exp Neurol 47:9–18

    Google Scholar 

  21. Nukina N, Ihara Y (1983) Immunocytochemical study on senile plaques in Alzheimer's disease. I. Preparation of an anti-microtubule-associated proteins (MAPs) antiserum and its specificity. Proc Jpn Acad [B] 59:284–287

    Google Scholar 

  22. 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) 44:211–216

    Google Scholar 

  23. Schmidt ML, Carden MJ, Lee M-Y, Trojanowski JQ (1987) Phosphate-dependent and-independent neurofilament epitopes in the axonal swellings of patients with motor neuron disease and controls. Lab Invest 56:282–294

    Google Scholar 

  24. Takahashi K, Nakamura H, Okada E (1972) Hereditary amyotrophic lateral sclerosis. Histochemical and electron microscopic study of hyaline inclusions in motor neurons. Arch Neurol 27:292–299

    Google Scholar 

  25. Tanaka S, Yase Y, Yoshimasu H (1980) Familial amyotrophic lateral sclerosis. Ultrastructural study of intraneuronal hyaline inclusion material. Adv Neurol Sci 24:386–387 [abstr]

    Google Scholar 

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Sasaki, S., Yamane, K., Sakuma, H. et al. Sporadic motor neuron disease with Lewy body-like hyaline inclusions. Acta Neuropathol 78, 555–560 (1989). https://doi.org/10.1007/BF00687719

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  • DOI: https://doi.org/10.1007/BF00687719

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