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Which ante mortem clinical features predict progressive supranuclear palsy pathology?

, ; Respondek, Gesine; Kurz, Carolin; Arzberger, Thomas; Compta, Yaroslau; Englund, Elisabet LU ; Ferguson, Leslie W.; Gelpi, Ellen; Giese, Armin and Irwin, David J., et al. (2017) In Movement Disorders 32(7). p.995-1005
Abstract

Background: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. Objective: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. Methods: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls and calculated the sensitivity, specificity, and positive predictive value of key clinical features for PSP in... (More)

Background: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. Objective: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. Methods: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls and calculated the sensitivity, specificity, and positive predictive value of key clinical features for PSP in this cohort. Results: Of 4166 articles identified by the database inquiry, 269 met predefined standards. The literature review identified clinical features predictive of PSP, including features of the following 4 functional domains: ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction. No biomarker or genetic feature was found reliably validated to predict definite PSP. High-quality original natural history data were available from 206 patients with pathologically diagnosed PSP and from 231 pathologically diagnosed disease controls (54 corticobasal degeneration, 51 multiple system atrophy with predominant parkinsonism, 53 Parkinson's disease, 73 behavioral variant frontotemporal dementia). We identified clinical features that predicted PSP pathology, including phenotypes other than Richardson's syndrome, with varying sensitivity and specificity. Conclusions: Our results highlight the clinical variability of PSP and the high prevalence of phenotypes other than Richardson's syndrome. The features of variant phenotypes with high specificity and sensitivity should serve to optimize clinical diagnosis of PSP.

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Contribution to journal
publication status
published
subject
keywords
clinical features, clinico-pathological series, diagnosis, Progressive supranuclear palsy, systematic review
in
Movement Disorders
volume
32
issue
7
pages
11 pages
publisher
John Wiley & Sons
external identifiers
  • scopus:85018897534
ISSN
0885-3185
DOI
10.1002/mds.27034
language
English
LU publication?
yes
id
b296c66f-644c-4166-9b2b-2a8601dd1232
date added to LUP
2019-06-29 22:54:40
date last changed
2019-09-17 04:57:47
@article{b296c66f-644c-4166-9b2b-2a8601dd1232,
  abstract     = {<p>Background: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. Objective: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. Methods: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP and relevant disease controls and calculated the sensitivity, specificity, and positive predictive value of key clinical features for PSP in this cohort. Results: Of 4166 articles identified by the database inquiry, 269 met predefined standards. The literature review identified clinical features predictive of PSP, including features of the following 4 functional domains: ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction. No biomarker or genetic feature was found reliably validated to predict definite PSP. High-quality original natural history data were available from 206 patients with pathologically diagnosed PSP and from 231 pathologically diagnosed disease controls (54 corticobasal degeneration, 51 multiple system atrophy with predominant parkinsonism, 53 Parkinson's disease, 73 behavioral variant frontotemporal dementia). We identified clinical features that predicted PSP pathology, including phenotypes other than Richardson's syndrome, with varying sensitivity and specificity. Conclusions: Our results highlight the clinical variability of PSP and the high prevalence of phenotypes other than Richardson's syndrome. The features of variant phenotypes with high specificity and sensitivity should serve to optimize clinical diagnosis of PSP.</p>},
  author       = {,  and Respondek, Gesine and Kurz, Carolin and Arzberger, Thomas and Compta, Yaroslau and Englund, Elisabet and Ferguson, Leslie W. and Gelpi, Ellen and Giese, Armin and Irwin, David J. and Meissner, Wassilios G. and Nilsson, Christer and Pantelyat, Alexander and Rajput, Alex and van Swieten, John C. and Troakes, Claire and Josephs, Keith A. and Lang, Anthony E. and Mollenhauer, Brit and Müller, Ulrich and Whitwell, Jennifer L. and Antonini, Angelo and Bhatia, Kailash P. and Bordelon, Yvette and Corvol, Jean Christophe and Colosimo, Carlo and Dodel, Richard and Grossman, Murray and Kassubek, Jan and Krismer, Florian and Levin, Johannes and Lorenzl, Stefan and Morris, Huw and Nestor, Peter and Oertel, Wolfgang H. and Rabinovici, Gil D. and Rowe, James B. and van Eimeren, Thilo and Wenning, Gregor K. and Boxer, Adam and Golbe, Lawrence I. and Litvan, Irene and Stamelou, Maria and Höglinger, Günter U.},
  issn         = {0885-3185},
  keyword      = {clinical features,clinico-pathological series,diagnosis,Progressive supranuclear palsy,systematic review},
  language     = {eng},
  month        = {07},
  number       = {7},
  pages        = {995--1005},
  publisher    = {John Wiley & Sons},
  series       = {Movement Disorders},
  title        = {Which ante mortem clinical features predict progressive supranuclear palsy pathology?},
  url          = {http://dx.doi.org/10.1002/mds.27034},
  volume       = {32},
  year         = {2017},
}