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Clinical diagnosis of progressive supranuclear palsy : The movement disorder society criteria

Höglinger, Günter U; Respondek, Gesine; Stamelou, Maria; Kurz, Carolin; Josephs, Keith A; Lang, Anthony E; Mollenhauer, Brit; Müller, Ulrich; Nilsson, Christer LU and Whitwell, Jennifer L., et al. (2017) In Movement Disorders 32(6). p.853-864
Abstract

Background: PSP is a neuropathologically defined disease entity. Clinical diagnostic criteria, published in 1996 by the National Institute of Neurological Disorders and Stroke/Society for PSP, have excellent specificity, but their sensitivity is limited for variant PSP syndromes with presentations other than Richardson's syndrome. Objective: We aimed to provide an evidence- and consensus-based revision of the clinical diagnostic criteria for PSP. Methods: We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for articles published in English since 1996, using postmortem diagnosis or highly specific clinical criteria as the diagnostic standard. Second, we generated retrospective standardized clinical data from patients with... (More)

Background: PSP is a neuropathologically defined disease entity. Clinical diagnostic criteria, published in 1996 by the National Institute of Neurological Disorders and Stroke/Society for PSP, have excellent specificity, but their sensitivity is limited for variant PSP syndromes with presentations other than Richardson's syndrome. Objective: We aimed to provide an evidence- and consensus-based revision of the clinical diagnostic criteria for PSP. Methods: We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for articles published in English since 1996, using postmortem diagnosis or highly specific clinical criteria as the diagnostic standard. Second, we generated retrospective standardized clinical data from patients with autopsy-confirmed PSP and control diseases. On this basis, diagnostic criteria were drafted, optimized in two modified Delphi evaluations, submitted to structured discussions with consensus procedures during a 2-day meeting, and refined in three further Delphi rounds. Results: Defined clinical, imaging, laboratory, and genetic findings serve as mandatory basic features, mandatory exclusion criteria, or context-dependent exclusion criteria. We identified four functional domains (ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction) as clinical predictors of PSP. Within each of these domains, we propose three clinical features that contribute different levels of diagnostic certainty. Specific combinations of these features define the diagnostic criteria, stratified by three degrees of diagnostic certainty (probable PSP, possible PSP, and suggestive of PSP). Clinical clues and imaging findings represent supportive features. Conclusions: Here, we present new criteria aimed to optimize early, sensitive, and specific clinical diagnosis of PSP on the basis of currently available evidence.

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publication status
published
subject
keywords
Clinical diagnostic criteria, Consensus-based, Evidence-based, Progressive supranuclear palsy
in
Movement Disorders
volume
32
issue
6
pages
853 - 864
publisher
John Wiley & Sons
external identifiers
  • scopus:85018960596
  • wos:000402969400010
ISSN
0885-3185
DOI
10.1002/mds.26987
language
English
LU publication?
yes
id
3695e927-88b5-425b-8028-8f2527a8d8b2
date added to LUP
2017-06-07 10:18:34
date last changed
2018-10-14 04:40:00
@article{3695e927-88b5-425b-8028-8f2527a8d8b2,
  abstract     = {<p>Background: PSP is a neuropathologically defined disease entity. Clinical diagnostic criteria, published in 1996 by the National Institute of Neurological Disorders and Stroke/Society for PSP, have excellent specificity, but their sensitivity is limited for variant PSP syndromes with presentations other than Richardson's syndrome. Objective: We aimed to provide an evidence- and consensus-based revision of the clinical diagnostic criteria for PSP. Methods: We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for articles published in English since 1996, using postmortem diagnosis or highly specific clinical criteria as the diagnostic standard. Second, we generated retrospective standardized clinical data from patients with autopsy-confirmed PSP and control diseases. On this basis, diagnostic criteria were drafted, optimized in two modified Delphi evaluations, submitted to structured discussions with consensus procedures during a 2-day meeting, and refined in three further Delphi rounds. Results: Defined clinical, imaging, laboratory, and genetic findings serve as mandatory basic features, mandatory exclusion criteria, or context-dependent exclusion criteria. We identified four functional domains (ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction) as clinical predictors of PSP. Within each of these domains, we propose three clinical features that contribute different levels of diagnostic certainty. Specific combinations of these features define the diagnostic criteria, stratified by three degrees of diagnostic certainty (probable PSP, possible PSP, and suggestive of PSP). Clinical clues and imaging findings represent supportive features. Conclusions: Here, we present new criteria aimed to optimize early, sensitive, and specific clinical diagnosis of PSP on the basis of currently available evidence.</p>},
  author       = {Höglinger, Günter U and Respondek, Gesine and Stamelou, Maria and Kurz, Carolin and Josephs, Keith A and Lang, Anthony E and Mollenhauer, Brit and Müller, Ulrich and Nilsson, Christer and Whitwell, Jennifer L. and Arzberger, Thomas and Englund, Elisabet and Gelpi, Ellen and Giese, Armin and Irwin, David J. and Meissner, Wassilios G. and Pantelyat, Alexander and Rajput, Alex and van Swieten, John C and Troakes, Claire and Antonini, Angelo and Bhatia, Kailash P. and Bordelon, Yvette and Compta, Yaroslau and Corvol, Jean-Christophe and Colosimo, Carlo and Dickson, Dennis W and Dodel, Richard and Ferguson, Leslie and Grossman, Murray and Kassubek, Jan and Krismer, Florian and Levin, Johannes and Lorenzl, Stefan and Morris, Huw R and Nestor, Peter and Oertel, Wolfgang H and Poewe, Werner and Rabinovici, Gil D. and Rowe, James B and Schellenberg, Gerard D. and Seppi, Klaus and van Eimeren, Thilo and Wenning, Gregor K. and Boxer, Adam L. and Golbe, Lawrence I. and Litvan, Irene},
  issn         = {0885-3185},
  keyword      = {Clinical diagnostic criteria,Consensus-based,Evidence-based,Progressive supranuclear palsy},
  language     = {eng},
  number       = {6},
  pages        = {853--864},
  publisher    = {John Wiley & Sons},
  series       = {Movement Disorders},
  title        = {Clinical diagnosis of progressive supranuclear palsy : The movement disorder society criteria},
  url          = {http://dx.doi.org/10.1002/mds.26987},
  volume       = {32},
  year         = {2017},
}