Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies

Research output: Contribution to journalArticle


Background: The Movement Disorder Society criteria for progressive supranuclear palsy introduced the category “probable 4-repeat (4R)-tauopathy” for joint clinical diagnosis of progressive supranuclear palsy and corticobasal degeneration. Objectives: To validate the accuracy of these clinical criteria for “probable 4R-tauopathy” to predict underlying 4R-tauopathy pathology. Methods: Diagnostic accuracy for 4R-tauopathies according to the established criteria was estimated retrospectively in autopsy-confirmed patients with progressive supranuclear palsy and corticobasal degeneration (grouped as 4R-tauopathies), and Parkinson's disease, multiple system atrophy, and frontotemporal lobar degeneration (grouped as non-4R-tauopathies). Results: We identified 250 cases with progressive supranuclear palsy (N = 195) and corticobasal degeneration (N = 55) and with and non-4R-tauopathies (N = 161). Sensitivity and specificity of “probable 4R-tauopathy” was 10% and 99% in the first year and 59% and 88% at final record. Conclusions: The new diagnostic category “probable 4R-tauopathy” showed high specificity and may be suitable for the recruitment of patients with progressive supranuclear palsy and corticobasal degeneration into therapeutic trials targeting 4R-tauopathy. The low sensitivity underpins the need for diagnostic biomarkers.


  • Gesine Respondek
  • Max Joseph Grimm
  • Ines Piot
  • Thomas Arzberger
  • Yaroslau Compta
  • Elisabet Englund
  • Leslie W. Ferguson
  • Ellen Gelpi
  • Sigrun Roeber
  • Armin Giese
  • Murray Grossman
  • David J. Irwin
  • Wassilios G. Meissner
  • Christer Nilsson
  • Alexander Pantelyat
  • Alex Rajput
  • John C. van Swieten
  • Claire Troakes
  • Günter U. Höglinger
  • for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group
External organisations
  • Technical University of Munich
  • German Center for Neurodegenerative Diseases (DZNE), Bonn
  • Ludwig-Maximilian University of Munich
  • University of Barcelona
  • Royal University Hospital, Saskatoon
  • University of Pennsylvania
  • University of Bordeaux
  • Groupe Hospitalier Pellegrin
  • University of Otago
  • Johns Hopkins University
  • Erasmus University Medical Center
  • King's College London
  • Hannover Medical School
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Neurosciences


  • corticobasal degeneration, diagnostic criteria, Four-repeat tauopathies, progressive supranuclear palsy
Original languageEnglish
JournalMovement Disorders
Publication statusE-pub ahead of print - 2019 Sep 30
Publication categoryResearch