Skip to main content
Log in

Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations

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

Abstract

We have studied brain tissues from three patients with corticobasal degeneration (CBD) histologically, ultrastructurally and immunohistochemically. Ballooned neurons in the cerebral cortex and severe degeneration of the substantia nigra were observed in them all and weakly basophilic neurofibrillary tangles (NFTs) were distributed widely in the basal ganglia and brain stem. Ultrastructural examination demonstrated that the NFTs comprised characteristic 15-nm-wide straight tubules, which showed positive immunohistochemical staining with an antibody against tau, but not ubiquitin. Tau-immunoreactive neuronal cell bodies without NFTs also were found in the cerebral cortex and subcortical nuclei, predominantly in the brain stem, and the greatest number of tau-positive glial inclusions occurred in the cerebral gray and white matter of the pre- and post-central gyri. These inclusions comprised tubular structures with diameters of about 15 nm and were localized in the oligodendroglial cellular cytoplasm and processes. These findings indicate that there is a close cytoskeletal pathological relationship between CBD and progressive supranuclear palsy.

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. Akashi T, Arima K, Maruyama N, Ando S, Inose T (1989) Severe cerebral atrophy in progressive supranuclear palsy: a case report. Clin Neuropathol 4: 195–199

    Google Scholar 

  2. Arima K, Murayama S, Oyanagi S, Akashi T, Inose T (1992) Presenile dementia with progressive supranuclear palsy tangles and Pick bodies: an unusual degenerative disorder involving the cerebral cortex, cerebral nuclei, and brain stem nuclei. Acta Neuropathol 84: 128–134

    Google Scholar 

  3. Bancher C, Lassmann H, Budka H, Grundke-Iqbal I, Iqbal K, Wiche G, Seitelberger F, Wisniewski HM (1987) Neurofibrillary tangles in Alzheimer's disease and progressive supranuclear palsy: antigenic similarities and differences. Microtubule-associated protein tau antigenicity is prominent in all types of tangles. Acta Neuropathol (Berl) 74: 39–46

    Google Scholar 

  4. Braak H, Braak E (1989) Cortical and subcortical argyrophilic grains characterize a disease associated with adult onset dementia. Neuropathol App Neurobiol 15: 13–26

    Google Scholar 

  5. Case Records of the Massachusetts General Hospital (1985) Case 38-1985. N Engl J Med 313: 739–748

    Google Scholar 

  6. Cruz-Sanchez FF, Rossi ML, Cardozo A, Deacon P, Tolosa E (1992) Clinical and pathological study of two patients with progressive supranuclear palsy and Alzheimer's changes. Antigenic determinants that distinguish cortical and subcortical neurofibrillary tangles. Neorosci Lett 136: 43–46

    Google Scholar 

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

    Google Scholar 

  8. Eidelberg D, Dhawan V, Moeller JR, Sidtis JJ, Ginos JZ, Strother SC, Cederbaum J, Greene P, Fahn S, Powers JM, Rottenberg DA (1991) The metabolic landscape of corticobasal ganglionic degeneration: regional asymmetries studied with positron emission tomography. J Neurol Neurosurg Psychiatry 54: 856–862

    Google Scholar 

  9. Ghatak NR, Nochlin D, Hadfield MG (1980) Neurofibrillary pathology in progressive supranuclear palsy. Acta Neuropathol (Berl) 52: 73–76

    Google Scholar 

  10. Gibb WRG, Luthert PJ, Marsden CD (1989) Corticobasal degeneration. Brain 112: 1171–1192

    Google Scholar 

  11. Hirano A, Dembitzer HM, Kurland LT, Zimmerman HM (1968) The fine structure of some intraganglionic alterations. Neurofibrillary tangles, granulovacuolar bodies and “rod-like” structures as seen in Guam amyotrophic lateral sclerosis and parkinsonism-dementia complex. J Neuropathol Exp Neurol 27: 167–182

    Google Scholar 

  12. Hsu S-M, Raine L, Fanger H (1981) Use of avidin-biotinperoxidase complex (ABC) in immunoperoxidase techniques: a comparison between ABC and unlabeled antibody (PAP) procedures. J Histochem Cytochem 29: 577–580

    Google Scholar 

  13. Ihara Y (1988) Massive somatodendritic sprouting of cortical neurons in Alzheimer's disease. Brain Res 459: 138–144

    Google Scholar 

  14. Jellinger K (1971) Progressive supranuclear palsy (subcortical argyrophilic dystrophy). Acta Neuropathol (Berl) 19: 347–352

    Google Scholar 

  15. Kageshita T, Nakashima T, Morinaga S, Tsuburaya M, Shimosato Y, Ishihara K, Arao T (1985) Immunohistochemical double staining using the avidin-biotin-alkaline phosphatase complex method. Pathol Clin Med (Tokyo) 3: 803–805

    Google Scholar 

  16. Kato S, Hirano A, Umahara T, Kato M, Herz F, Ohama E (1992) Comparative immunohistochemical study on the expression of αB crystallin, ubiquitin and stress-response protein 27 in ballooned neurons in various disorders. Neuropathol Appl Neurobiol 18: 335–340

    Google Scholar 

  17. Kato S, Hirano A, Umahara T, Llena JF, Herz F, Ohama E (1992) Ultrastructural and immunohistochemical studies on ballooned cortical neurons in Creutzfeldt-Jakob disease: expression of αB-crystallin, ubiquitin and stress-response protein 27. Acta Neuropathol 84: 443–448

    Google Scholar 

  18. Lippa CF, Smith TW, Fountneau N (1990) Corticonigral degeneration with neuronal achromasia. A clinicopathologic study of two cases. J Neurol Sci 98: 301–310

    Google Scholar 

  19. Mitani K, Uchihara T, Tamaru F, Endo K, Tsukagoshi H (1993) Corticobasal degeneration: clinico-pathological studies on two cases. Clin Neurol (Tokyo) 33: 155–161

    Google Scholar 

  20. Nishimura M, Namba Y, Ikeda K, Oda M (1992) Glial fibrillary tangles with straight tubules in the brains of patients with progressive supranuclear palsy. Neurosci Lett 143: 35–38

    Google Scholar 

  21. Nukina N, Quan Y, Nakano I, Otomo E (1992) Widespread tau abnormality in a case of corticobasal degeneration. Clin Neurol (Tokyo) 32: 1093–1101

    Google Scholar 

  22. Oyanagi K, Takahashi H, Wakabayashi K, Ikuta F (1988) Selective decrease of large neurons in the neostriatum in progressive supranuclear palsy. Brain Res 458: 218–223

    Google Scholar 

  23. Oyanagi K, Takahashi H, Wakabayashi K Ikuta F (1991) Large neurons in the neostriatum in Alzheimer's disease and progressive supranuclear palsy: a topographic, histologic and ultrastructural investigation. Brain Res 544: 221–226

    Google Scholar 

  24. Oyanagi S (1974) On the ultrastructure of the aging structure of the brain (I). Brain Nerve (Tokyo) 26: 637–653

    Google Scholar 

  25. Paulus W, Selim M (1990) Corticonigral degeneration with neuronal achromasia and basal neurofibrillary tangles. Acta Neuropathol 81: 89–94

    Google Scholar 

  26. Rebeiz JJ, Kolodny EH, Richardson EP (1968) Corticodentatonigral degeneration with neuronal achromasia. Arch Neurol 18: 20–33

    Google Scholar 

  27. Riley DE, Lang AE, Lewis A, Resch L, Ashby P, Hornykiewiez O, Black S (1990) Cortical-basal ganglionic degeneration. Neurology 40: 1203–1212

    Google Scholar 

  28. Sako H, Nakamura H, Inose K, Takada K, Tanaka J, Tabuchi Y (1986) Progressive supranuclear palsy. A case with a marked frontal atrophy. Neuropathology 7: 7–14

    Google Scholar 

  29. Smith TW, Lippa CF, de Girolami U (1992) Immunocytochemical study of ballooned neurons in cortical degeneration with neuronal achromasia. Clin Neuropathol 11: 28–35

    Google Scholar 

  30. Steele JC (1972) Progressive supranuclear palsy. Brain 95: 693–704

    Google Scholar 

  31. Steele JC, Richardson JC, Olszewski J (1964) Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 10: 333–359

    Google Scholar 

  32. Takahashi H, Takeda S, Ikuta F, Homma Y (1987) Progressive supranuclear palsy with limbic system involvement: report of a case with ultrastructural investigation of neurofibrillary tangles in various locations. Clin Neuropathol 6: 271–276

    Google Scholar 

  33. Takahashi H, Oyanagi K, Takeda S, Hinokuma K, Ikuta F (1989) Occurrence of 15-nm-wide straight tubules in neocortical neurons in progressive supranuclear palsy. Acta Neuropathol 79: 233–239

    Google Scholar 

  34. Tellez-Nagel I, Wisniewski HM (1973) Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski syndrome. Arch Neurol 29: 324–327

    Google Scholar 

  35. Tomonaga M (1977) Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropathol (Berl) 37: 177–181

    Google Scholar 

  36. Yamada T, McGeer PL (1990) Oligodendroglial microtubular masses: an abnormality observed in some human neurodegenerative diseases. Neurosci Lett 120: 163–166

    Google Scholar 

  37. Yamada T, McGeer PL, McGeer EG (1992) Appearance of paired nucleated, Tau-positive glia in patients with progressive supranuclear palsy brain tissue. Neurosci Lett 135: 99–102

    Google Scholar 

  38. Yamada T, Calne DB, Akiyama H, McGeer EG, McGeer PL (1993) Further observations on Tau-positive glia in the brains with progressive supranuclear palsy. Acta Neuropathol 85: 308–315

    Google Scholar 

  39. Yamamoto T, Kawamura J, Hashimoto S, Nakamura M, Iwamoto H, Kobashi Y, Ichijima K (1990) Pallido-nigro-luysian atrophy, progressive supranuclear palsy and adult onset Hallervorden-Spatz disease: a case of akinesia as a predominant feature of parkinsonism. J Neurol Sci 101: 98–106

    Google Scholar 

  40. Zweig RM, Whitehouse PJ, Casanova MF, Walker LC, Jankel WR, Price DL (1987) Loss of pedunculopontine neurons in progressive supranuclear palsy. Ann Neurol 22: 18–25

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wakabayashi, K., Oyanagi, K., Makifuchi, T. et al. Corticobasal degeneration: etiopathological significance of the cytoskeletal alterations. Acta Neuropathol 87, 545–553 (1994). https://doi.org/10.1007/BF00293314

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00293314

Key words

Navigation