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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)

Abstract

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.

Keywords

Tyrosine Hydroxylase Progressive Supranuclear Palsy Neurofibrillary Tangle Progressive Supranuclear Palsy Senile Dementia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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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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