Abstract |
Postencephalitic parkinsonism (PEP), a chronic complication of encephalitis lethargica, is a tauopathy characterized by multisystem neuronal loss and gliosis with widespread neurofibrillary lesions composed of both 3- and 4-repeat (3R and 4R) tau isoforms. Previous immunohistochemical studies in a small number of PEP cases demonstrated absence of Lewy bodies as well as the lack of other alpha-synuclein pathology, classifying PEP as a "pure" tauopathy. Neuropathologic examination of 10 brains with clinico-pathologically verified PEP confirmed widespread neurodegeneration in subcortical and brainstem areas associated with multifocal neurofibrillary pathology comprising both 3R and 4R tau. Very rare beta-amyloid deposits were observed in two elderly patients, while Lewy bodies and neurites or any other alpha-synuclein deposits were completely absent. The causes and molecular background of total absence of alpha-synuclein pathology in PEP, in contrast to most other tauopathies, remain as unknown as the pathogenesis of PEP.
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Authors | Kurt A Jellinger |
Journal | Acta neuropathologica
(Acta Neuropathol)
Vol. 118
Issue 3
Pg. 371-9
(Sep 2009)
ISSN: 1432-0533 [Electronic] Germany |
PMID | 19404653
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Amyloid beta-Peptides
- alpha-Synuclein
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Topics |
- Adult
- Amyloid beta-Peptides
(metabolism)
- Brain
(metabolism, pathology)
- Brain Stem
(metabolism, pathology)
- Female
- Humans
- Immunohistochemistry
- Male
- Middle Aged
- Nerve Degeneration
- Neurons
(metabolism)
- Parkinson Disease, Postencephalitic
(metabolism, pathology)
- Tauopathies
(metabolism, pathology)
- alpha-Synuclein
(metabolism)
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