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

  • Author(s): Respondek, Gesine
  • Grimm, Max-Joseph
  • Piot, Ines
  • Arzberger, Thomas
  • Compta, Yaroslau
  • Englund, Elisabet
  • Ferguson, Leslie W
  • Gelpi, Ellen
  • Roeber, Sigrun
  • Giese, Armin
  • Grossman, Murray
  • Irwin, David J
  • Meissner, Wassilios G
  • Nilsson, Christer
  • Pantelyat, Alexander
  • Rajput, Alex
  • van Swieten, John C
  • Troakes, Claire
  • Höglinger, Günter U
  • Movement Disorder Society-Endorsed Progressive Supranuclear Palsy Study Group
  • et al.


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.


To validate the accuracy of these clinical criteria for "probable 4R-tauopathy" to predict underlying 4R-tauopathy pathology.


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).


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.


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. © 2019 International Parkinson and Movement Disorder Society.

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