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Neurofibrillary pathology in progressive supranuclear palsy (PSP)
J Cervós-Navarro1, K Schumacher
1Institute of Neuropathology, Free University of Berlin, Federal Republic of Germany.
Journal of Neural Transmission. Supplementum
|January 1, 1994
Summary
Progressive supranuclear palsy (PSP) involves neurofibrillary tangles (NFT) in the brain, distinct from Alzheimer's disease. These changes suggest non-specific cytoskeletal disorganization in PSP.
Area of Science:
- Neuropathology
- Neurodegenerative Diseases
Background:
- Progressive supranuclear palsy (PSP) is characterized by neurofibrillary tangles (NFT).
- The relationship between neuronal loss and NFT presence in PSP is inverse.
- Neurologic findings in PSP have been linked to carcinomas as a paraneoplastic effect.
Purpose of the Study:
- To investigate the nature of neuritic changes in PSP.
- To compare these changes with those found in Alzheimer's disease.
- To explore the underlying molecular mechanisms of cytoskeletal disorganization in PSP.
Main Methods:
- Histopathological examination of globose neurofibrillary tangles (NFT) in PSP.
- Immunohistochemistry using antibodies against tau and ubiquitin.
- Comparison of filament structures in PSP and Alzheimer's disease.
Main Results:
- Globose NFT are found in subcortical areas of PSP brains.
- Neuritic changes in PSP basal ganglia consist of straight filaments and tubules.
- Immunohistochemistry shows antigenic profiles similar to early Alzheimer's disease NFT.
Conclusions:
- PSP involves unique straight filaments, distinct from Alzheimer's paired helical filaments.
- These findings suggest a novel fibrous protein in PSP neuritic changes.
- The alterations in PSP support a hypothesis of non-specific cytoskeletal disorganization.