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Pyramidal system involvement in progressive supranuclear palsy - a clinicopathological correlation
Z. Stejskalova, Z. Rohan, R. Rusina, A. Tesar, J. Kukal, GG. Kovacs, A. Bartos, R. Matej,
Language English Country England, Great Britain
Document type Journal Article
Grant support
VFN64165
Ministerstvo Zdravotnictví Ceské Republiky
TN64190
Thomayer hospital
Q27/LF1
Univerzita Karlova v Praze
Q28/LF1
Univerzita Karlova v Praze
Q35/LF3
Univerzita Karlova v Praze
GAUK 113115
Univerzita Karlova v Praze
NPU I LO1611
Ministerstvo Školství, Mládeže a Tělovýchovy
AVASTipendium
Czech Alzheimer Foundation and AVAST!
NLK
BioMedCentral
from 2001-12-01
BioMedCentral Open Access
from 2001
Directory of Open Access Journals
from 2001
Free Medical Journals
from 2001
PubMed Central
from 2001
Europe PubMed Central
from 2001
ProQuest Central
from 2009-01-01
Open Access Digital Library
from 2001-01-01
Open Access Digital Library
from 2001-01-01
Open Access Digital Library
from 2001-01-01
Medline Complete (EBSCOhost)
from 2001-12-18
Health & Medicine (ProQuest)
from 2009-01-01
ROAD: Directory of Open Access Scholarly Resources
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Springer Nature OA/Free Journals
from 2001-12-01
- MeSH
- Immunohistochemistry MeSH
- Middle Aged MeSH
- Humans MeSH
- Cerebral Cortex pathology MeSH
- Parkinsonian Disorders diagnosis pathology MeSH
- Movement Disorders diagnosis pathology MeSH
- Supranuclear Palsy, Progressive diagnosis pathology MeSH
- tau Proteins metabolism MeSH
- Pyramidal Tracts pathology MeSH
- Retrospective Studies MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged, 80 and over MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
BACKGROUND: We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP). METHODS: Pyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson's syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Clinical manifestations of motor neuron involvement (pseudobulbar or bulbar signs and spasticity) were retrospectively assessed semiquantitatively. Neuropathologically, hyperphosphorylated tau-related pyramidal motor system neuronal, neuritic, and glial pathology using anti-tau AT8 clone immunohistochemistry, was also evaluated. RESULTS: Clinical manifestations of pyramidal motor system involvement were found in patients with different PSP subtypes. A statistically significant higher load of tau pathology was found in the pyramidal system in PSP-Richardson's syndrome compared to other PSP subtypes (p = 0.016); however, there was no significant correlation between pyramidal system tau pathology and related motor clinical symptoms. CONCLUSIONS: Tau pathology in the spinal cord and pyramidal motor system structures is very common in progressive supranuclear palsy and may neuropathologically supplement the distinction between classic Richardson's syndrome from other progressive supranuclear palsy subtypes.
References provided by Crossref.org
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- $a Stejskalova, Zuzana $u Department of Pathology and Molecular Medicine Third Faculty of Medicine, Charles University and Thomayer Hospital, Videnska 800, 14059, Prague 4 - Krc, Czech Republic. Department of Pathology, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic. Department of Pathology, Third Faculty of Medicine, Charles University and University Hospital Kralovske Vinohrady, Prague, Czech Republic.
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- $a Pyramidal system involvement in progressive supranuclear palsy - a clinicopathological correlation / $c Z. Stejskalova, Z. Rohan, R. Rusina, A. Tesar, J. Kukal, GG. Kovacs, A. Bartos, R. Matej,
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- $a BACKGROUND: We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP). METHODS: Pyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson's syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Clinical manifestations of motor neuron involvement (pseudobulbar or bulbar signs and spasticity) were retrospectively assessed semiquantitatively. Neuropathologically, hyperphosphorylated tau-related pyramidal motor system neuronal, neuritic, and glial pathology using anti-tau AT8 clone immunohistochemistry, was also evaluated. RESULTS: Clinical manifestations of pyramidal motor system involvement were found in patients with different PSP subtypes. A statistically significant higher load of tau pathology was found in the pyramidal system in PSP-Richardson's syndrome compared to other PSP subtypes (p = 0.016); however, there was no significant correlation between pyramidal system tau pathology and related motor clinical symptoms. CONCLUSIONS: Tau pathology in the spinal cord and pyramidal motor system structures is very common in progressive supranuclear palsy and may neuropathologically supplement the distinction between classic Richardson's syndrome from other progressive supranuclear palsy subtypes.
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