Chameleons and mimics: Progressive supranuclear palsy and corticobasal degeneration.
4-repeat tauopathy
astrocytc plaque
corticobasal degeneration
progressive supranuclear palsy
tufted astrocyte
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Feb 2020
Feb 2020
Historique:
received:
28
06
2019
revised:
05
07
2019
accepted:
05
07
2019
pubmed:
14
9
2019
medline:
13
11
2020
entrez:
14
9
2019
Statut:
ppublish
Résumé
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are neurodegenerative disorders that show parkinsonism as their main symptom. Both PSP and CBD are sporadic tauopathies associated with hyperphosphorylated four-repeat tau aggregation in neurons and glial cells. The characteristic pathologies of PSP are midbrain atrophy and the appearance of tufted astrocytes and globose-type neurofibrillary tangles. PSP shows severe degeneration in the globus pallidus, substantia nigra, subthalamic nucleus, and cerebellar dentate nuclei. Conversely, the characteristic pathologies of CBD are cortical atrophy and the appearance of astrocytic plaques and argyrophilic threads. CBD is associated with severe degeneration in the cerebral white matter, substantia nigra, and globus pallidus. Clinical symptoms depend on the topographical distribution and severity of degeneration rather than on the type of aggregated protein or inclusions. PSP and CBD present clinically differential diagnostic difficulties because of their overlapping pathological distributions.
Substances chimiques
MAPT protein, human
0
tau Proteins
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
57-67Subventions
Organisme : AMED
ID : JP16kk0205009
Organisme : AMED
ID : JP18dm0107105
Informations de copyright
© 2019 Japanese Society of Neuropathology.
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