Validation of the movement disorder society criteria for the diagnosis of 4‐repeat tauopathies. Issue 1 (30th September 2019)

• Record Type: Journal Article
• Authors: 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.
• Other Names: Aiba Ikuko investigator.; Antonini Angelo investigator.; Arzberger Thomas investigator.; Barone Paolo investigator.; Bhatia Kailash P. investigator.; Boxer Adam K. investigator.; Colosimo Carlo investigator.; Compta Yaroslau investigator.; Corvol Jean Christophe investigator.; Dickson Dennis W. investigator.; Gelpi Ellen investigator.; Giese Armin investigator.; Golbe Lawrence I. investigator.; Grossman Murray investigator.; Höglinger Günter U. investigator.; Hopfner Franziska investigator.; Irwin David J. investigator.; Josephs Keith A. investigator.; Kassubek Jan investigator.; Kovacs Gabor G. investigator.; Lang Anthony E. investigator.; Levin Johannes investigator.; Litvan Irene investigator.; Höllerhage Matthias investigator.; McFarland Nikolaus investigator.; Meissner Wassilios G. investigator.; Morris Huw R. investigator.; Müller Ulrich investigator.; Nilsson Christer investigator.; Oertel Wolfgang H. investigator.; Pantelyat Alexander investigator.; Rajput Alex investigator.; Respondek Gesine investigator.; Rowe James B. investigator.; Sakakibara Ruji investigator.; Schellenberg Gerard investigator.; Stamelou Maria investigator.; Troakes Claire investigator.; van Eimeren Thilo investigator.; van Swieten John C. investigator.; Wenning Gregor K. investigator.; Whitwell Jennifer L. investigator.;
• Is Part Of: Movement disorders. Volume 35:Issue 1(2020)
• Page Start: 171
• Page End: 176
• Publication Date: 2019-09-30
• Subjects: corticobasal degeneration -- diagnostic criteria -- Four‐repeat tauopathies -- progressive supranuclear palsy; Movement disorders -- Periodicals; 610
• DOI: 10.1002/mds.27872
• Languages: English
• ISSNs: 0885-3185

Abstract

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. © 2019 International Parkinson and Movement Disorder Society