@article{be167c71d135407995f5e9b115e9c42a,
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 = "Funding Information: Gesine Respondek has received honoraria for speaker‐related activities from Biogen. Max‐Joseph Grimm has nothing to disclose.Ines Piot has nothing to disclose. Thomas Arzberger has nothing to disclose. Yaroslau Compta: has received funding, research support and/or honoraria from the EC H2020 and the CERCA Programme of Generalitat de Catalunya, UCB, Teva, Medtronic, Abbvie, Merz, Piramal Imaging and Alter. Elisabet Englund has nothing to disclose. Leslie W. Ferguson has nothing to disclose. Ellen Gelpi has received research support from the Fundaci{\'o} Marat{\'o} de TV3 (grant n° 20141610), and Fundaci{\'o}n Tatiana P{\'e}rez de Guzm{\'a}n del Bueno; has served on the advisory board of the ICM Institute for Brain and Spinal Cord, Hopital Piti{\'e} Salpetri{\'e}re, Paris, France, and the Oxford Parkinson Disease Center, Oxford, UK.Sigrun Roeber has nothing to disclose. Armin Giese holds stocks and shares in and has personal links with MODAG. He also holds shares in the patent “EP2307381” and diverse national secondary patents.Murray Grossman has nothing to disclose.David J. Irwin is supported by National Institutes of Health grant K23 NS088341. Wassilios G. Meissner has received fees for editorial activities with Springer, has served as advisor for Sanofi, Lundbeck and Affiris, has received teaching honoraria from TEVA, UCB and Aguettant, as well as research support from the Michael J Fox Foundation, the University Hospital Bordeaux, the French Health Ministry, the European Community, ANR, ARAMISE, PSP‐France, MSA Coalition, LABEX Excellence Initiative. Christer Nilsson: has received research support from the Swedish Alzheimer Fund, Sk{\aa}ne University Hospital grants and the Swedish Research Council ALF grants. Alexander Pantelyat was supported by NIH grant K23 AG059891. Alex Rajput has received research support from the Regina Curling Classic, Greystone Classic for Parkinson's, Inc. and the Dr. Ali Rajput Endowment for Parkinson's Disease and Movement Disorders; has been co‐investigator on grant funded by International Essential Tremor Foundation (IETF) (study period 2012‐2013); has received research funding as principal investigator for the clinical study by Teva, protocol no.: TVP‐1012/501 (Aug/09 – June/13); has received speaker and travel honoraria from Teva, Allergan and the Parkinson Society Canada.John C. van Swieten has nothing to disclose.Claire Troakes has nothing to disclose.G{\"u}nter U. H{\"o}glinger has served on the advisory boards for AbbVie, Alzprotect, Asceneuron, Biogen, Novartis, Roche, Sanofi, UCB; has received honoraria for scientific presentations from Abbvie, Biogen, Roche, Teva, UCB, has received research support from CurePSP, the German Academic Exchange Service (DAAD), German Parkinson's Disease Foundation (DPG), German PSP Association (PSP Gesellschaft), German Research Foundation (DFG) and the German Ministry of Education and Research (BMBF), International Parkinson's Fonds (IPF); has received institutional support from the German Center for Neurodegenerative Diseases (DZNE). Publisher Copyright: {\textcopyright} 2019 International Parkinson and Movement Disorder Society",
year = "2020",
month = jan,
day = "1",
doi = "10.1002/mds.27872",
language = "English (US)",
volume = "35",
pages = "171--176",
journal = "Movement Disorders",
issn = "0885-3185",
publisher = "John Wiley and Sons Inc.",
number = "1",
}