Journal Article

DZNE-2022-01385

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Long-duration progressive supranuclear palsy: clinical course and pathological underpinnings.

Jecmenica Lukic, M. (First author)DZNE* ; Respondek, G.Extern* ; Kurz, C.DZNE* ; Compta, Y. ; Gelpi, E. ; Ferguson, L. W. ; Rajput, A. ; Troakes, C. ; group, M. P. s. (Collaboration Author) ; van Swieten, J. C. ; Giese, A.Extern* ; Roeber, S. ; Herms, J.DZNE* ; Arzberger, T.DZNE* ; Höglinger, G. (Last author)DZNE* ; Giese, A. ; Troakes, C. ; Gelpi, E. ; Respondek, G.Extern* ; Höglinger, G. U.Extern* ; van Swieten, J. ; Arzberger, T.Extern* ; Compta, Y. ; Molina-Porcel, L. ; Aldecoa, I. ; Tolosa, E. ; Martí, M. J. ; Valldeoriola, F. ; Pagonabarraga, J. ; Calopa, M. ; Bayès, A. ; Hernandez, I. ; Aguilar, M. ; Genis, D.

2022
Wiley-Blackwell Hoboken, NJ

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Please use a persistent id in citations: doi:10.1002/ana.26455

Abstract: To identify the clinical characteristics of the subgroup of benign progressive supranuclear palsy with particularly long disease duration; to define neuropathological determinants underlying variability in disease duration in progressive supranuclear palsy.Clinical and pathological features were compared among 186 autopsy-confirmed cases with progressive supranuclear palsy with ≥10 years and shorter survival times.45 cases (24.2%) had a disease duration of ≥10 years. The absence of ocular motor abnormalities within the first 3 years from disease onset was the only significant independent clinical predictor of longer survival. Histopathologically, the neurodegeneration parameters in each survival group were paralleled anatomically by the distribution of neuronal cytoplasmic inclusions, whereas the tufted astrocytes displayed anatomically an opposite severity pattern. Most interestingly, we found significantly less coiled bodies in those who survive longer, in contrast to patients with less favorable course.A considerable proportion of patients had a more 'benign' disease course with ≥10 years survival. They had a distinct pattern and evolution of core symptoms compared to patients with short survival. The inverted anatomical patterns of astrocytic tau distribution suggest distinct implications of these cell types in trans-cellular propagation. The tempo of disease progression appeared to be determined mostly by oligodendroglial tau, where high degree of oligodendroglial tau pathology might affect neuronal integrity and function on top of neuronal tau pathology. The relative contribution of glial tau should be further explored in cellular and animal models. This article is protected by copyright. All rights reserved.

Keyword(s): Astrocytes: metabolism (MeSH) ; Autopsy (MeSH) ; Disease Progression (MeSH) ; Humans (MeSH) ; Neurons: metabolism (MeSH) ; Supranuclear Palsy, Progressive: pathology (MeSH) ; tau Proteins: metabolism (MeSH)

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Contributing Institute(s):

1. Coordinator of Clinical Parkinson Research (AG Höglinger 2)
2. Neuropathology / Brainbank (Neuropathology / Brainbank)
3. Translational Brain Research (AG Herms)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)
2. 352 - Disease Mechanisms (POF4-352) (POF4-352)

Appears in the scientific report 2022

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Database coverage:
; ; ; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; Current Contents - Life Sciences ; DEAL Wiley ; Ebsco Academic Search ; Essential Science Indicators ; IF >= 10 ; JCR ; Nationallizenz ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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