Advertisement

Journal of Neurology

, Volume 239, Issue 8, pp 426–430 | Cite as

Ubiquitin-positive intraneuronal inclusions in the extramotor cortices of presenile dementia patients with motor neuron disease

  • Koichi Okamoto
  • Nobuyuki Murakami
  • Hirofumi Kusaka
  • Mari Yoshida
  • Yoshio Hashizume
  • Yoichi Nakazato
  • Etsuro Matsubara
  • Shunsaku Hirai
Original Communications

Summary

Ubiquitin-positive intraneuronal inclusions were found in the extramotor cortices of ten presenile dementia patients with motor neuron disease. There were inclusions in the hippocampal granular cells and in the small neurons of the superficial layers of the temporal and frontal cortices. Bunina bodies were present in the anterior horn cells in all cases. These results suggest that ubiquitin-related cytoskeletal abnormalities are common in cerebral non-motor small neurons in these patients.

Key words

Motor neuron disease Dementia Ubiquitin Amyotrophic lateral sclerosis 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Dalakas MC, Hatazawa J, Brooks RA, Di Chiro G (1987) Lowered cerebral glucose utilization in amyotrophic lateral sclerosis. Ann Neurol 22:580–586Google Scholar
  2. 2.
    Gallassi R, Montagna P, Ciardulli C, Lorusso S, Mussuto V, Stracciari A (1985) Cognitive impairment in motor neuron disease. Acta Neurol Scand 71:480–484Google Scholar
  3. 3.
    Horoupian DS, Thal L, Katzman R, Terry RD, Davies P, Hirano A, DeTeresa R, Fuld PA, Petito C, Blass J, Ellis JM (1984) Dementia and motor neuron disease: morphometric, biochemical, and Golgi studies. Ann Neurol 16:305–313Google Scholar
  4. 4.
    Hudson AJ (1981) Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain 104:217–247Google Scholar
  5. 5.
    Knopman DS, Mastri AR, Frey WH, Sung JH, Rustan T (1990) Dementia lacking distinctive histologic features: a common non-Alzheimer degenerative dementia. Neurology 40:251–256Google Scholar
  6. 6.
    Leigh PN, Anderton BH, Dodson A, Gallo J-M, Swash M, Power DM (1988) Ubiquitin deposits in anterior horn cells in motor neurone disease. Neurosci Lett 93:197–203Google Scholar
  7. 7.
    Love S, Saitoh T, Quijada S, Terry RD (1988) Alz-50, ubiquitin and tau immunoreactivity of neurofibrillary tangles, Pick bodies and Lewy bodies. J Neuropathol Exp Neurol 47:393–405Google Scholar
  8. 8.
    Lowe J, Blanchard A, Morrell K, Lennox G, Reynolds L, Billett M, Landon M, Mayer RJ (1988) Ubiquitin is a common factor in intermediate filament inclusion bodies or diverse type in man, including those of Parkinson's disease, Pick's disease, and Alzheimer's disease, as well as Rosenthal fibers in cerebellar astrocytomas, cytoplasmic bodies in muscle, and Mallory bodies in alcoholic liver disease. J Pathol 155:9–15Google Scholar
  9. 9.
    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 neurones in motor neurone disease defined by immunocytochemical localisation of ubiquitin. Neurosci Lett 94:203–210Google Scholar
  10. 10.
    Lowe J, Aldridge F, Lennox G, Doherty F, Jefferson D, Landon M, Mayer RJ (1989) Inclusion bodies in motor cortex and brainstem of patients with motor neurone disease detected by immunocytochemical localisation of ubiquitin. Neurosci Lett 105:7–13Google Scholar
  11. 11.
    Ludolph AC, Elger CE, Böttger IW, Kuttig AG, Lottes G, Brune GG (1987) N-isoipropyl-p-123I-amphetamine single photon emission computer tomography in motor neuron disease. Eur Neurol 29:255–260Google Scholar
  12. 12.
    Manetto V, Perry G, Tabaton M, Mulvihill P, Fried VA, Smith HT, Gambetti P, Autilio-Gambetti L (1988) Ubiquitin is associated with abnormal cytoplasmic filaments characteristic of neurodegenerative diseases. Proc Natl Acad Sci USA 85:4501–4505Google Scholar
  13. 13.
    Migheli A, Autilio-Gambetti L, Gambetti P, Mocellini C, Vigliani MC, Schiffer D (1990) Ubiquitinated filamentous inclusions in spinal cord of patients with motor neuron disease. Neurosci Lett 114:5–10Google Scholar
  14. 14.
    Mitsuyama Y (1984) Presenile dementia with motor neuron disease in Japan: clinico-pathological review of 26 cases. J Neurol Neurosurg Psychiatry 47:953–959Google Scholar
  15. 15.
    Morita K, Kaiya H, Ikeda T, Namba M (1987) Presenile dementia combined with amyotrophy: a review of 34 Japanese cases. Arch Gerontol Geriatr 6:263–277Google Scholar
  16. 16.
    Murayama S, Mori H, Ihara Y, Bouldin TW, Suzuki K, Tomonaga M (1990) Immunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis. Ann Neurol 27:137–148Google Scholar
  17. 17.
    Neary D, Snowden JS, Mann DMA, Northen B, Goulding PJ, Macdermott N (1990) Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry 53:23–32Google Scholar
  18. 18.
    Nihei K, McKee AC, Kowall NW (1991) Ubiquitin immunoreactivity in the Rolandic cortex of patients with sporadic and familial amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 50:310Google Scholar
  19. 19.
    Okamoto K, Hirai S, Shoji M, Harigaya Y, Fukuda T (1991) Widely distributed Bunina bodies and spheroids in a case of atypical sporadic amyotrophic lateral sclerosis. Acta Neuropathol 81:349–353Google Scholar
  20. 20.
    Okamoto K, Hirai S, Yamazaki T, Sun X, Nakazato Y (1991) New Ubquitin-positive intraneuronal inclusions in the extramotor cortices in patients with amyotrophic lateral sclerosis. Neurosci Lett 129:233–236Google Scholar
  21. 21.
    Salazar AM, Masters CL, Gadjusek DC, Gipps CJ Jr (1983) Syndrome of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease. Ann Neurol 14:17–26Google Scholar

Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • Koichi Okamoto
    • 1
  • Nobuyuki Murakami
    • 3
  • Hirofumi Kusaka
    • 4
  • Mari Yoshida
    • 3
  • Yoshio Hashizume
    • 5
  • Yoichi Nakazato
    • 2
  • Etsuro Matsubara
    • 2
  • Shunsaku Hirai
    • 1
  1. 1.Department of NeurologyGunma University School of MedicineGunmaJapan
  2. 2.Department of PathologyGunma University School of MedicineGunmaJapan
  3. 3.Department of NeurologyHigashi Nagoya National HospitalNagoyaJapan
  4. 4.Department of NeurologyKitano HospitalOsakaJapan
  5. 5.Department of PathologyNagoya University School of MedicineNagoyaJapan

Personalised recommendations