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

Research output: Contribution to journalArticle

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.

Details

Authors
  • 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
  • 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 D. Rabinovici
  • James B Rowe
  • Gerard D. Schellenberg
  • Klaus Seppi
  • Thilo van Eimeren
  • Gregor K. Wenning
  • Adam L. Boxer
  • Lawrence I. Golbe
  • Irene Litvan
Organisations
External organisations
  • German Center for Neurodegenerative Diseases (DZNE)
  • Mayo Clinic Minnesota
  • Justus Liebig University Giessen
  • Hospital Clínic of Barcelona
  • University of Pennsylvania
  • Groupe Hospitalier Pellegrin
  • University of Baltimore
  • Royal University Hospital, Saskatoon
  • Erasmus University Medical Center
  • King's College London
  • Veneto Institute of Oncology
  • University of Barcelona
  • Pitié-Salpêtrière University Hospital
  • Mayo Clinic Florida
  • University Hospital Essen
  • University Hospital Munich
  • University College London
  • German Center for Neurodegenerative Diseases (DZNE) Magdeburg
  • Philipp University of Marburg
  • University of Texas Health Science Centre
  • University of Cambridge
  • National and Kapodistrian University of Athens
  • Ludwig-Maximilian University of Munich
  • Toronto Western Hospital
  • University Medical Center Göttingen
  • World Orthopaedic Concern
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Neurology

Keywords

  • Clinical diagnostic criteria, Consensus-based, Evidence-based, Progressive supranuclear palsy
Original languageEnglish
Pages (from-to)853-864
JournalMovement Disorders
Volume32
Issue number6
Early online date2017 Apr
Publication statusPublished - 2017
Publication categoryResearch
Peer-reviewedYes