Progressive Supranuclear Palsy: Clinico-Pathological and Biochemical Studies

  • K. Jellinger
  • P. Riederer
  • M. Tomonaga
Part of the Journal of Neural Transmission book series (NEURAL SUPPL, volume 16)


Ten autopsy eases of Progressive Supranuclear Palsy (PSP) are reported. Age at onset ranged from 16 to 67 years and the duration of illness 3 to 24 years. The clinical features were aggressive mental retardation in 4 cases with early onset, paroxysmal dysequilibrium, ophthalmoplegia, rigidity and akinesia, pseudobulbär palsy and variable degrees of dementia. Neuropathology showed widespread neurofibrillary degeneration associated with system-bound neuronal loss and gliosis in subcortical areas, particularly affecting the subthalamic nucleus, substantia nigra, brainstem tegmentum and dentate nuclei, with no or little involvement of the cerebral cortex. The distribution of the lesions and the ultrastructure of the neurofibrillary tangles made of 15 nm straight filaments (seen in one case) in PSP are different from postencephalitic parkinsonism, Guam Parkinson-dementia complex and brainstem affection in (pre)senile dementia. Post-mortem biochemical analysis of two brains disclosed severe reduction of tyrosine hydroxylase, the key synthetic enzyme of the catecholamine pathway, not only in the nigrostriatal system as seen in Parkinson’s disease, but in most areas of the brainstem and limbic system. The implication and possible pathogenic and therapeutic significance of these biochemical findings are discussed. The etiology of PSP and its nosological position within the degenerative extrapyramidal disorders remain unknown.


Tyrosine Hydroxylase Progressive Supranuclear Palsy Neurofibrillary Tangle Progressive Supranuclear Palsy Senile Dementia 
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  1. Albert, M. L., Feldman, R. G., Willis, A. L.: “The subcortical dementia” of progressive supranuclear palsy. J. Neurol. Neurosurg. Psychiat. 37, 121–130 (1974).PubMedCrossRefGoogle Scholar
  2. Barbeau, A.: Degenerescence plurissystematisee du nevrauxe. Syndrome de Steele-Richardson-Olszewski. Un. med. Canada 94, 715–718 (1965).PubMedGoogle Scholar
  3. Birkmayer, W., Danielczyk, W., Neumayer, E., Riederer, P.: Nucleus ruber and L-Dopa psychosis. Biochemical post-mortem findings. J. Neural Transm. 35, 93–116 (1974).PubMedCrossRefGoogle Scholar
  4. Birkmayer, W., Riederer, P.: Responsibility of extrastriatal areas for the appearance of psychotic symptoms. J. Neural Transm. 37, 175–182 (1975).PubMedCrossRefGoogle Scholar
  5. Bugiani, L., Mancardi, G. L., Brusa, A., Ederli, E.; The fine structure of subcortical neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropath. (Berlin) 45, 147–152 (1979).CrossRefGoogle Scholar
  6. Colmant, H.: Progressive supranuclear palsy. Arch. Psychiat. Nervenkr. 214, 324–330 (1971).PubMedCrossRefGoogle Scholar
  7. Constantinidis, J., Tissot, R., De Ajuriaguerra, J.: Dystonie oculo-facio- cervicale ou paralysie progressive supranucleaire de Steele-Richardson- Olszewski. Rev. neurol. 122, 249–262 (1970).PubMedGoogle Scholar
  8. Hirano, A., Malamud, N., Elizan, T. S., Kurland, L. T.: Amyotrophic lateral sclerosis and Parkinson-dementia complex on Guam. Arch. Neurol. (Chic.) 15, 35–51 (1966).CrossRefGoogle Scholar
  9. Ishii, Y., Itoh, T.: An autopsy case of progressive supranuclear palsy. Clin. Neurol. (Tokyo) 19, 187 (1979).CrossRefGoogle Scholar
  10. Ishino, H., Ikeda, H., Otsuki, S.: Contribution to the clinical pathology of progressive supranuclear palsy (subcortical argyrophilic dystrophy). J. Neurol. Sci. 24, 471–481 (1975).PubMedCrossRefGoogle Scholar
  11. Jellinger, K.: Progressive supranuclear palsy (subcortical argyrophilic dystrophy). Acta Neuropath. (Berlin) 19, 347–352 (1971).CrossRefGoogle Scholar
  12. Klawans, H. L., Ringel, S. P.; Observations on the efficacy of L-Dopa in progressive supranuclear palsy. Europ. Neurol. 5, 115–129 (1975).CrossRefGoogle Scholar
  13. Kurihara, T., Landau, W. M., Torack, R. M.: Progressive supranuclear palsy with actions myoclonus, seizures. Neurology (Minneap.) 24, 219–223 (1971).Google Scholar
  14. Mastaglia, F. L., Grainger, K. M. R.: Internuclear opthalmoplegia in progressive supranuclear palsy. J. Neurol. Sci. 25, 303–308 (1975).PubMedCrossRefGoogle Scholar
  15. McGeer, E. G., Gibson, S., McGeer, P. L.: Some characteristics of brain tyrosine hydroxylase. Canad. J. Biochem. 45, 1557–1563 (1967).CrossRefGoogle Scholar
  16. McGeer, P. L., Hattori, T., Singh, V. K., McGeer, E. G.: Cholinergic symptoms on extrapyramidal function. In: Basal Ganglia (Ahr, M. D., ed.), pp. 213–222. New York: Plenum Press. 1976.Google Scholar
  17. Mendeil, J. R., Chase, T. N., Engel, W. K.: Modifikation by L-Dopa of a case of progressive supranuclear palsy. With evidence of defective cerebral dopamine metabolism. Lancet I, 593–594 (1970).CrossRefGoogle Scholar
  18. Morax, P. U., Aron-Rosa, D., Barthold, I., Contamin, F., Mignot, M.: Les paralysies supranucleaires progressives. Ann. Oculist (Paris) 207, 267 to 278 (1974).Google Scholar
  19. Nagatsu, T., Kato, T., Numata, Y., Ikuta, K., Sano, M., et al.: Phenyl- ethanolamine-N-methyl transferase and other enzymes of catecholamine metabolism in human brain. Clin. Chim. Acta 75, 221–232 (1977).PubMedCrossRefGoogle Scholar
  20. Owen, F., Gross, A.J., Crow, T.J., Reynolds, G., Riederer, P.: In preparation.Google Scholar
  21. Perkin, G. D., Lees, A.J., Stern, G. M., Kolen, R. S.: Problems in the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski Syndrome). Canad. J. Neurol. Sci. 5, 167–173 (1978).Google Scholar
  22. Powell, H. C., London, G. W., Lantpert, P. W.: Neurofibrillary tangles in progressive supranuclear palsy. Electron microscopic observations. J. Neuropath. exp. Neurol. 33, 98–106 (1974).PubMedCrossRefGoogle Scholar
  23. Probst, A.: Dégénérescence de neurofibrillaire sous-corticale senile avec presence de tubule contournes et de filaments droits. Forme atypique de la paralysie supranucleaire progressive. Rev. Neurol. 133, 417–428 (1977).PubMedGoogle Scholar
  24. Probst, A., Dufresne, J.,: Paralysie supranucleaire progressive ou dystonie oculo-facio-cervicale. Arch. Suisses Neurol. Neurochir. Psychiat. 116, 107–134 (1975).Google Scholar
  25. Riederer, P., Rausch, W. D., Birkmayer, W., Jellinger, K., Seemann, D.: CNS modulation of adrenal tyrosine hydroxylase in Parkinson’s disease and metabolic eneephalopathies. J. Neural Transm., Suppl. 14, 121–131. Wien-New York: Springer. 1978.Google Scholar
  26. Riederer, P., Wuketich, S.: Time course of nigrostriatal degeneration in Parkinson’s disease. J. Neural Transm. 38, 277–301 (1976).PubMedCrossRefGoogle Scholar
  27. Rouzaud, M., Degiovanni, E., Jobard, P., Gray, E., Durand, J. P.: L’ophthalmoplegie supranucleaire progressive (Syndrome de Steele- Richardson-Olszewski). Rev. Neurol. 130, 143–164 (1974).PubMedGoogle Scholar
  28. Roy, S., Datta, C. K., Hirano, A., Ghatak, N. R., Zimmerman, H. M.: Electron microscopic study of neurofibrillary tangles in Steele- Richardson-Olszewski Syndrome. Acta Neuropath. (Berlin) 29, 175 to 179 (1974).CrossRefGoogle Scholar
  29. Seitelb erger, F: Heterogenous system degeneration. Subcortical argyrophilic dystrophy. Acta Neurol. (Napoli) 24, 276–284 (1969).Google Scholar
  30. Steele, J. C.: Progressive supranuclear palsy. Brain 95, 693–704 (1972).PubMedGoogle Scholar
  31. Steele, J. C., Richardson, J. C., Olszewski, J.: Progressive supranuclear palsy. Arch. Neurol. (Chic.) 10, 333–359 (1964).CrossRefGoogle Scholar
  32. Stevens, J. R., Livermore, A., jr.: Kindling of the mesolimbic dopamine system: Animal model of psychosis. Neurology (Minneap.) 28, 36–46 (1978).Google Scholar
  33. Tellez-Nagel, I., Wisniewski, H. M.: Ultrastructure of neurofibrillary tangles in Steele-Richardson-Olszewski Syndrome. Arch. Neurol. (Chic.) 29, 324–327 (1973).CrossRefGoogle Scholar
  34. Tomonaga, M.: Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropath. (Berlin) 37, 177–181 (1977).CrossRefGoogle Scholar
  35. Troost, B. T., Daroff, R. B.: The ocular motor defects in progressive supranuclear palsy. Ann. Neurol. 2, 397–403 (1977).PubMedCrossRefGoogle Scholar
  36. Yagishita, S., Itoh, Y., Amano, N., Nakano, T., Saitoh, A.: Ultrastructure of neurofibrillary tangles in progressive supranuclear palsy. Acta Neuropath. (Berlin) 48, 27–30 (1979).Google Scholar
  37. Wisniewski, H. M., Soffer, D.: Neurofibrillary pathology: current status and research perspectives. Mech. Ageing Developm. 9, 119–142 (1979).CrossRefGoogle Scholar

Copyright information

© Springer-Verlag 1980

Authors and Affiliations

  • K. Jellinger
    • 1
    • 2
    • 3
  • P. Riederer
    • 1
    • 2
  • M. Tomonaga
    • 1
    • 2
  1. 1.Ludwig Boltzmann-Institute of Clinical NeurobiologyLainz-HospitalWienAustria
  2. 2.Department of Clinical Pathology (Neuropathology)Tokyo Metropolitan Institute of GerontologyTokyoJapan
  3. 3.Ludwig Boltzmann-Institute of Clinical NeurobiologyLainz-HospitalWienAustria

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