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Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center

BACKGROUND/OBJECTIVES: The clinical diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remains challenging due to heterogeneity of the diseases. AIMS: Here we compared the clinical features of PSP and MSA to gain insight into their diagnosis and prognosis, particular...

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Autores principales: Xie, Tao, Kang, Un J, Kuo, Sheng-Han, Poulopoulos, Markos, Greene, Paul, Fahn, Stanley
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5516563/
https://www.ncbi.nlm.nih.gov/pubmed/28725681
http://dx.doi.org/10.1038/npjparkd.2015.7
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author Xie, Tao
Kang, Un J
Kuo, Sheng-Han
Poulopoulos, Markos
Greene, Paul
Fahn, Stanley
author_facet Xie, Tao
Kang, Un J
Kuo, Sheng-Han
Poulopoulos, Markos
Greene, Paul
Fahn, Stanley
author_sort Xie, Tao
collection PubMed
description BACKGROUND/OBJECTIVES: The clinical diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remains challenging due to heterogeneity of the diseases. AIMS: Here we compared the clinical features of PSP and MSA to gain insight into their diagnosis and prognosis, particularly the prognostic value of down-gaze palsy latency in PSP progression. METHODS: We reviewed clinical features of pathologically confirmed 10 PSP and 13 MSA patients, incidentally matched in age-at-onset, gender, and disease duration, followed at Columbia University Medical Center during 1994–2009. RESULTS: The final antemortem diagnosis was incorrect in 30% of PSP (all lacking down-gaze palsy) and 23% of MSA patients. Falls in the first year of the disease, pyramidal involvement and freezing of gait during the course were similar between PSP and MSA. Ataxia and apraxia were in 50% of the PSP patients. Parkinsonism responsive to levodopa treatment was in 30% of the PSP (all with resting tremor) and 50% of the MSA patients. Dysautonomia in MSA could occur as early as 3 years preceding the motor symptoms, with 46% within the first year of the motor onset, but 15% did not have dysautonomia in life. The latency of down-gaze palsy and urogenital dysfunction and MMSE scores at first visit in PSP, and the latency of falls and wheelchair confinement in MSA were all associated with the disease progression. CONCLUSIONS: We confirmed most of the previously published characterizations, and identified for the first time the prognostic value of down-gaze palsy latency in PSP progression.
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spelling pubmed-55165632017-07-19 Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center Xie, Tao Kang, Un J Kuo, Sheng-Han Poulopoulos, Markos Greene, Paul Fahn, Stanley NPJ Parkinsons Dis Article BACKGROUND/OBJECTIVES: The clinical diagnosis of progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remains challenging due to heterogeneity of the diseases. AIMS: Here we compared the clinical features of PSP and MSA to gain insight into their diagnosis and prognosis, particularly the prognostic value of down-gaze palsy latency in PSP progression. METHODS: We reviewed clinical features of pathologically confirmed 10 PSP and 13 MSA patients, incidentally matched in age-at-onset, gender, and disease duration, followed at Columbia University Medical Center during 1994–2009. RESULTS: The final antemortem diagnosis was incorrect in 30% of PSP (all lacking down-gaze palsy) and 23% of MSA patients. Falls in the first year of the disease, pyramidal involvement and freezing of gait during the course were similar between PSP and MSA. Ataxia and apraxia were in 50% of the PSP patients. Parkinsonism responsive to levodopa treatment was in 30% of the PSP (all with resting tremor) and 50% of the MSA patients. Dysautonomia in MSA could occur as early as 3 years preceding the motor symptoms, with 46% within the first year of the motor onset, but 15% did not have dysautonomia in life. The latency of down-gaze palsy and urogenital dysfunction and MMSE scores at first visit in PSP, and the latency of falls and wheelchair confinement in MSA were all associated with the disease progression. CONCLUSIONS: We confirmed most of the previously published characterizations, and identified for the first time the prognostic value of down-gaze palsy latency in PSP progression. Nature Publishing Group 2015-05-21 /pmc/articles/PMC5516563/ /pubmed/28725681 http://dx.doi.org/10.1038/npjparkd.2015.7 Text en Copyright © 2015 Parkinson's Disease Foundation/Macmillan Publishers Limited http://creativecommons.org/licenses/by/4.0/ This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
spellingShingle Article
Xie, Tao
Kang, Un J
Kuo, Sheng-Han
Poulopoulos, Markos
Greene, Paul
Fahn, Stanley
Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center
title Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center
title_full Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center
title_fullStr Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center
title_full_unstemmed Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center
title_short Comparison of clinical features in pathologically confirmed PSP and MSA patients followed at a tertiary center
title_sort comparison of clinical features in pathologically confirmed psp and msa patients followed at a tertiary center
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5516563/
https://www.ncbi.nlm.nih.gov/pubmed/28725681
http://dx.doi.org/10.1038/npjparkd.2015.7
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