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Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation

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 neuropat...

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Autores principales: Stejskalova, Zuzana, Rohan, Zdenek, Rusina, Robert, Tesar, Adam, Kukal, Jaromir, Kovacs, Gabor G., Bartos, Ales, Matej, Radoslav
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425568/
https://www.ncbi.nlm.nih.gov/pubmed/30894142
http://dx.doi.org/10.1186/s12883-019-1270-1
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author Stejskalova, Zuzana
Rohan, Zdenek
Rusina, Robert
Tesar, Adam
Kukal, Jaromir
Kovacs, Gabor G.
Bartos, Ales
Matej, Radoslav
author_facet Stejskalova, Zuzana
Rohan, Zdenek
Rusina, Robert
Tesar, Adam
Kukal, Jaromir
Kovacs, Gabor G.
Bartos, Ales
Matej, Radoslav
author_sort Stejskalova, Zuzana
collection PubMed
description 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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12883-019-1270-1) contains supplementary material, which is available to authorized users.
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spelling pubmed-64255682019-03-29 Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation Stejskalova, Zuzana Rohan, Zdenek Rusina, Robert Tesar, Adam Kukal, Jaromir Kovacs, Gabor G. Bartos, Ales Matej, Radoslav BMC Neurol Research Article 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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1186/s12883-019-1270-1) contains supplementary material, which is available to authorized users. BioMed Central 2019-03-20 /pmc/articles/PMC6425568/ /pubmed/30894142 http://dx.doi.org/10.1186/s12883-019-1270-1 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Stejskalova, Zuzana
Rohan, Zdenek
Rusina, Robert
Tesar, Adam
Kukal, Jaromir
Kovacs, Gabor G.
Bartos, Ales
Matej, Radoslav
Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
title Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
title_full Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
title_fullStr Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
title_full_unstemmed Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
title_short Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
title_sort pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425568/
https://www.ncbi.nlm.nih.gov/pubmed/30894142
http://dx.doi.org/10.1186/s12883-019-1270-1
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