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

for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalMovement Disorders
DOIs
StateAccepted/In press - Jan 1 2019

Fingerprint

Tauopathies
Movement Disorders
Progressive Supranuclear Palsy
Frontotemporal Lobar Degeneration
Multiple System Atrophy
Patient Selection
Parkinson Disease
Autopsy
Joints
Biomarkers

Keywords

  • corticobasal degeneration
  • diagnostic criteria
  • Four-repeat tauopathies
  • progressive supranuclear palsy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group (Accepted/In press). Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies. Movement Disorders. https://doi.org/10.1002/mds.27872

Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies. / for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group.

In: Movement Disorders, 01.01.2019.

Research output: Contribution to journalArticle

for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group 2019, 'Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies', Movement Disorders. https://doi.org/10.1002/mds.27872
for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group. Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies. Movement Disorders. 2019 Jan 1. https://doi.org/10.1002/mds.27872
for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group. / Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies. In: Movement Disorders. 2019.
@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 = "corticobasal degeneration, diagnostic criteria, Four-repeat tauopathies, 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.}",
year = "2019",
month = "1",
day = "1",
doi = "10.1002/mds.27872",
language = "English (US)",
journal = "Movement Disorders",
issn = "0885-3185",
publisher = "John Wiley and Sons Inc.",

}

TY - JOUR

T1 - Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies

AU - for the Movement Disorder Society–Endorsed Progressive Supranuclear Palsy Study Group

AU - Respondek, Gesine

AU - Grimm, Max Joseph

AU - Piot, Ines

AU - Arzberger, Thomas

AU - Compta, Yaroslau

AU - Englund, Elisabet

AU - Ferguson, Leslie W.

AU - Gelpi, Ellen

AU - Roeber, Sigrun

AU - Giese, Armin

AU - Grossman, Murray

AU - Irwin, David J.

AU - Meissner, Wassilios G.

AU - Nilsson, Christer

AU - Pantelyat, Alexander

AU - Rajput, Alex

AU - van Swieten, John C.

AU - Troakes, Claire

AU - Höglinger, Günter U.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - corticobasal degeneration

KW - diagnostic criteria

KW - Four-repeat tauopathies

KW - progressive supranuclear palsy

UR - http://www.scopus.com/inward/record.url?scp=85073963867&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85073963867&partnerID=8YFLogxK

U2 - 10.1002/mds.27872

DO - 10.1002/mds.27872

M3 - Article

C2 - 31571273

AN - SCOPUS:85073963867

JO - Movement Disorders

JF - Movement Disorders

SN - 0885-3185

ER -