@article{2060b20e94d64bbfa984f73bdde9bf8d,
title = "Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies",
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 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.",
keywords = "Four-repeat tauopathies, corticobasal degeneration, diagnostic criteria, progressive supranuclear palsy",
author = "{for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group} and Gesine Respondek and Grimm, {Max Joseph} and Ines Piot and Thomas Arzberger and Yaroslau Compta and Elisabet Englund and Ferguson, {Leslie W.} and Ellen Gelpi and Sigrun Roeber and Armin Giese and Murray Grossman and Irwin, {David J.} and Meissner, {Wassilios G.} and Christer Nilsson and Alexander Pantelyat and Alex Rajput and {van Swieten}, {John C.} and Claire Troakes and H{\"o}glinger, {G{\"u}nter U.} and Ikuko Aiba and Angelo Antonini and Paolo Barone and Bhatia, {Kailash P.} and Boxer, {Adam K.} and Carlo Colosimo and Corvol, {Jean Christophe} and Dickson, {Dennis W.} and Golbe, {Lawrence I.} and Franziska Hopfner and Josephs, {Keith A.} and Jan Kassubek and Kovacs, {Gabor G.} and Lang, {Anthony E.} and Johannes Levin and Irene Litvan and Matthias H{\"o}llerhage and Nikolaus McFarland and Morris, {Huw R.} and Ulrich M{\"u}ller and Oertel, {Wolfgang H.} and Rowe, {James B.} and Ruji Sakakibara and Gerard Schellenberg and Maria Stamelou and {van Eimeren}, Thilo and Wenning, {Gregor K.} and Whitwell, {Jennifer L.}",
note = "Publisher Copyright: {\textcopyright} 2019 International Parkinson and Movement Disorder Society",
year = "2020",
month = jan,
day = "1",
doi = "10.1002/mds.27872",
language = "English",
volume = "35",
pages = "171--176",
journal = "Movement Disorders",
issn = "0885-3185",
publisher = "John Wiley & Sons Inc.",
number = "1",
}