Acta Neuropathologica

, Volume 19, Issue 4, pp 347–352 | Cite as

Progressive supranuclear palsy

Subcortical argyrophilic dystrophy
  • K. Jellinger
Short Original Communications

Summary

Two female cases of progressive supranuclear palsy (“heterogeneous system degeneration”) with histological findings are reported. Clinically, vertical gaze palsy, dystonia, akinesia, pseudobulbar palsy and mental impairment were prominent. Pathologically, widespread appearance of neurofibrillary tangles of the subcortical type in the brain stem and basal ganglia was associated with neuronal loss and gliosis in the substantia nigra, subthalamic nuclei and pallidum. There was less severe affection of the dentate nuclei and minimal cortical involvement. The distribution of changes in “subcortical argyrophilic dystrophy” is critically compared with and separated from brain stem affection in (pre)senile Alzheimer's disease.

Key words

Progressive Supranuclear Palsy Heterogenous System Degeneration Subcortical Argyrophilic Dystrophy Neurofibrillary Tangles Brain Stem 

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References

  1. 1.
    Anzil, A. P.: Progressive supranuclear palsy. Case report with pathological findings. Acta neuropath. (Berl.)14, 72–76 (1969).Google Scholar
  2. 2.
    Behrman, S., Carroll, J. D., Janota, I., Matthews, W. B.: Progressive supranuclear palsy. Clinico-pathological study of four cases. Brain92, 663–678 (1969).Google Scholar
  3. 3.
    Cambier, J., Escourolle, R., Masson, M., Lechevalier, B.: La paralysie supra-nucléaire progressive. A propos d'une observation anatomo-clinique. Rev. neurol.121, 139–154 (1969).Google Scholar
  4. 3a.
    Colmant, H. J.: Progressive supranuclear palsy. Arch. Psychiat. Nervenkr.214, 324–330 (1971).Google Scholar
  5. 4.
    Constantinidis, J., Tissot, R., de Ajuriaguerra, J.: Dystonie oculofacio-cervicale ou paralysie progressive supranucléaire de Steele-Richardson-Olszewski. Rev. neurol.122, 249–262 (1970).Google Scholar
  6. 5.
    Dehaene, I., Bogaerts, M.: La paralysie supranucléaire progressive. Acta neurol. belg.71, 141–151 (1971).Google Scholar
  7. 6.
    Dix, H. R., Harrison, M. J. G., Lewis, P. D.: Progressive supranuclear palsy (The Steele-Richardson-Olszewski syndrome). A report of 9 cases with particular reference to the mechanisms of the oculomotor disorders. J. neurol. Sci.13, 237–256 (1971).Google Scholar
  8. 7.
    Hirano, A., Malamud, N., Elizan, T. S., Kurland, L. T.: Amuotrophic lateral sclerosis and Parkinson-dementia complex on Guam. Arch. Neurol. (Chic.)15, 35–51 (1966).Google Scholar
  9. 8.
    —, Zimmerman, H. M.: Alzheimer's neurofibrillary changes. A topographic study. Arch. Neurol. (Chic.)7, 227–242 (1962).Google Scholar
  10. 9.
    Ishii, T.: Distribution of Alzheimer's neurofibrillary changes in the brain stem and hypothalamus of senile dementia. Acta neuropath. (Berl.)6, 181–187 (1966).Google Scholar
  11. 10.
    Jellinger, K.: Morphologische Gesichtspunkte zur Differenzierung sog. hirnatrophischer Prozesse. 1. Symposium A. G. für Gerontopsychiatrie. Krefeld, 8.–9. 5. 1971.Google Scholar
  12. 10a.
    Klawans, H. L., Jr., Ringel, S. P.: Observations on the efficacy ofl-Dopa in progressive supranuclear palsy. Europ. Neurol.5, 115–129 (1971).Google Scholar
  13. 11.
    Mehraein, P.: Argyrophilic dystrophies. Proc. IInd Int. Congr. Ass. Sci. Study Ment. Retard., Warszaw 1970.Google Scholar
  14. 12.
    Seitelberger, F.: Heterogenous system degeneration. Subcortical argyrophilic dystrophy. Acta neurol. (Bari)24, 276–284 (1969).Google Scholar
  15. 13.
    Stadlan, E. M., Duvoisin, R., Yahr, M.: The pathology of parkinsonism. Proc. VIth. Int. Congr. Neuropath., Exc. med., I. C. S. Nr. 100, pp. 569–571. Amsterdam-New York: Excerpta medica 1966.Google Scholar
  16. 14.
    Steele, J. C., Richardson, J. C., Olszewski, J.: Progressive supranuclear palsy. Arch. Neurol. (Chic.)10, 333–359 (1964).Google Scholar
  17. 15.
    Tariska, St.: Circumscribed cerebral atrophy in Alzheimer's disease. A pathological study. In: Alzheimer's disease and Related Conditions. G. E. W. Wolstenholme and M. O'Connor (ed.), pp. 51–73. London: Churchill 1970.Google Scholar

Copyright information

© Springer-Verlag 1971

Authors and Affiliations

  • K. Jellinger
    • 1
  1. 1.Neurological Institute of University of ViennaWienAustria

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