Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria
Günter U Höglinger, Gesine Respondek, Maria Stamelou, Carolin Kurz, Keith A Josephs, Anthony E Lang, Brit Mollenhauer, Ulrich Müller, Christer Nilsson, Jennifer L Whitwell, Thomas Arzberger, Elisabet Englund, Ellen Gelpi, Armin Giese, David J Irwin, Wassilios G Meissner, Alexander Pantelyat, Alex Rajput, John C van Swieten, Claire Troakes, Angelo Antonini, Kailash P Bhatia, Yvette Bordelon, Yaroslau Compta, Jean-Christophe Corvol, Carlo Colosimo, Dennis W Dickson, Richard Dodel, Leslie Ferguson, Murray Grossman, Jan Kassubek, Florian Krismer, Johannes Levin, Stefan Lorenzl, Huw R Morris, Peter Nestor, Wolfgang H Oertel, Werner Poewe, Gil Rabinovici, James B Rowe, Gerard D Schellenberg, Klaus Seppi, Thilo van Eimeren, Gregor K Wenning, Adam L Boxer, Lawrence I Golbe, Irene Litvan, Movement Disorder Society-endorsed PSP Study Group, Adam L Boxer, Alex Rajput, Alexander Pantelyat, Angelo Antonini, Anthony E Lang, Armin Giese, Brit Mollenhauer, Carlo Colosimo, Caroline Kurz, Christer Nilsson, Claire Troakes, David J Irwin, Dennis W Dickson, Ellen Gelpi, Florian Krismer, Gerard D Schellenberg, Gesine Respondek, Gil Rabinovici, Gregor K Wenning, Günter U Höglinger, Huw R Morris, Irene Litvan, James B Rowe, Jan Kassubek, Jean-Christophe Corvol, Jennifer L Whitwell, Johannes Levin, John van Swieten, Kailash P Bhatia, Keith A Josephs, Klaus Seppi, Lawrence I Golbe, Maria Stamelou, Murray Grossman, Peter Nestor, Richard Dodel, Stefan Lorenzl, Thilo van Eimeren, Thomas Arzberger, Ulrich Müller, Wassilios G Meissner, Werner Poewe, Wolfgang H Oertel, Yaroslau Compta, Yvette Bordelon, Günter U Höglinger, Gesine Respondek, Maria Stamelou, Carolin Kurz, Keith A Josephs, Anthony E Lang, Brit Mollenhauer, Ulrich Müller, Christer Nilsson, Jennifer L Whitwell, Thomas Arzberger, Elisabet Englund, Ellen Gelpi, Armin Giese, David J Irwin, Wassilios G Meissner, Alexander Pantelyat, Alex Rajput, John C van Swieten, Claire Troakes, Angelo Antonini, Kailash P Bhatia, Yvette Bordelon, Yaroslau Compta, Jean-Christophe Corvol, Carlo Colosimo, Dennis W Dickson, Richard Dodel, Leslie Ferguson, Murray Grossman, Jan Kassubek, Florian Krismer, Johannes Levin, Stefan Lorenzl, Huw R Morris, Peter Nestor, Wolfgang H Oertel, Werner Poewe, Gil Rabinovici, James B Rowe, Gerard D Schellenberg, Klaus Seppi, Thilo van Eimeren, Gregor K Wenning, Adam L Boxer, Lawrence I Golbe, Irene Litvan, Movement Disorder Society-endorsed PSP Study Group, Adam L Boxer, Alex Rajput, Alexander Pantelyat, Angelo Antonini, Anthony E Lang, Armin Giese, Brit Mollenhauer, Carlo Colosimo, Caroline Kurz, Christer Nilsson, Claire Troakes, David J Irwin, Dennis W Dickson, Ellen Gelpi, Florian Krismer, Gerard D Schellenberg, Gesine Respondek, Gil Rabinovici, Gregor K Wenning, Günter U Höglinger, Huw R Morris, Irene Litvan, James B Rowe, Jan Kassubek, Jean-Christophe Corvol, Jennifer L Whitwell, Johannes Levin, John van Swieten, Kailash P Bhatia, Keith A Josephs, Klaus Seppi, Lawrence I Golbe, Maria Stamelou, Murray Grossman, Peter Nestor, Richard Dodel, Stefan Lorenzl, Thilo van Eimeren, Thomas Arzberger, Ulrich Müller, Wassilios G Meissner, Werner Poewe, Wolfgang H Oertel, Yaroslau Compta, Yvette Bordelon
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 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. © 2017 International Parkinson and Movement Disorder Society.
Keywords: clinical diagnostic criteria; consensus-based; evidence-based; progressive supranuclear palsy.
Conflict of interest statement
Relevant conflicts of interest/financial disclosures: Nothing to report. Full financial disclosures and author roles may be found in the online version of this article.
© 2017 International Parkinson and Movement Disorder Society.
Source: PubMed