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Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies.
Authors
Thomas Arzberger
Yaroslau Compta
+17 more
Elisabet Englund
Leslie Ferguson
Ellen Gelpi
Armin Giese
Max-Joseph Grimm
Murray Grossman
Günter Höglinger
David Irwin
Wassilios Meissner
Christer Nilsson
Alexander Pantelyat
Ines Piot
Alex Rajput
Gesine Respondek
Sigrun Roeber
Claire Troakes
John van Swieten
Publication date
1 January 2020
Publisher
eScholarship, University of California
Abstract
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 Parkinsons 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. © 2019 International Parkinson and Movement Disorder Society
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Last time updated on 02/12/2023