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Mov Disord. 2019 Sep 30. doi: 10.1002/mds.27872. [Epub ahead of print]

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

Author information

1
Department of Neurology, Technische Universität München, Munich, Germany.
2
German Center for Neurodegenerative Diseases, Munich, Germany.
3
Department of Psychiatry and Psychotherapy, University Hospital, Ludwig-Maximilians-University Munich, Munich, Germany.
4
Center for Neuropathology and Prion Research, Ludwig-Maximilians-University Munich, Munich, Germany.
5
Parkinson's Disease & Movement Disorders Unit, Hospital Clínic/August Pi i Sunyer Biomedical Research Institute (IDIBAPS)/Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas(CIBERNED)/European Reference Network for Rare Neurological Diseases/Institut de Neurociències, Maeztu center, Universitat de Barcelona, Catalonia, Spain.
6
Department of Clinical Sciences, Division of Neurology, Lund University, Lund, Sweden.
7
Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatchewan, Canada.
8
Neurological Tissue Bank and Neurology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, IDIBAPS, Centres de Recerca de Catalunya (CERCA), Barcelona, Catalonia, Spain.
9
Institute of Neurology, Medical University of Vienna, Vienna, Austria.
10
Frontotemporal Degeneration Center, Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
11
Univ. de Bordeaux, Institut des Maladies Neurodégénératives, Unité Mixte de Recherche (UMR), 5293, 33000, Bordeaux, France.
12
Service de Neurologie, Hôpital Pellegrin, Centre Hospitalier Universitaire (CHU) de Bordeaux, 33000, Bordeaux, France.
13
Department of Medicine, University of Otago, Christchurch, New Zealand.
14
New Zealand Brain Research Institute, Christchurch, New Zealand.
15
Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
16
Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands.
17
London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry, Psychology and Neuroscience, Kings College London, London, UK.
18
Department of Neurology, Hanover Medical School, Hanover, Germany.

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.

KEYWORDS:

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

PMID:
31571273
DOI:
10.1002/mds.27872

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