Clinical Conditions “Suggestive of Progressive Supranuclear Palsy”—Diagnostic Performance

Max‐Joseph Grimm, Gesine Respondek, Maria Stamelou, Thomas Arzberger, Leslie Ferguson, Ellen Gelpi, Armin Giese, Murray Grossman, David J. Irwin, Alexander Pantelyat, Alex Rajput, Sigrun Roeber, John C. Swieten, Claire Troakes, Wassilios G. Meissner, Christer Nilsson, Ines Piot, Yaroslau Compta, James B. Rowe, Günter U. Höglinger,
Mov Disord. 2020-09-11; 35(12): 2301-2313
DOI: 10.1002/mds.28263

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Grimm MJ(1)(2), Respondek G(2)(3), Stamelou M(4)(5)(6), Arzberger T(2)(7)(8), Ferguson L(9), Gelpi E(10)(11), Giese A(7), Grossman M(12), Irwin DJ(12), Pantelyat A(13), Rajput A(9), Roeber S(7), van Swieten JC(14), Troakes C(15), Meissner WG(16)(17)(18), Nilsson C(19), Piot I(1)(2), Compta Y(20)(21)(22), Rowe JB(23), Höglinger GU(1)(2)(3); Movement Disorder Society-Endorsed PSP Study Group.

Author information:
(1)Department of Neurology, Technische Universität München, Munich, Germany.
(2)German Center for Neurodegenerative Diseases (DZNE), Munich, Germany.
(3)Department of Neurology, Hannover Medical School, Hannover, Germany.
(4)Parkinson’s Disease and Movement Disorders Department, HYGEIA Hospital,
National and Kapodistrian University of Athens, Athens, Greece.
(5)First Department of Neurology, Aiginiteion Hospital, National and Kapodistrian
University of Athens, Athens, Greece.
(6)Department of Neurology, Philipps Universität, Marburg, Germany.
(7)Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität,
Munich, Germany.
(8)Department of Psychiatry and Psychotherapy, University Hospital, LMU Munich,
Munich, Germany.
(9)Division of Neurology, Royal University Hospital, University of Saskatchewan,
Saskatoon, Saskatchewan, Canada.
(10)Neurological Tissue Bank and Neurology Department, Hospital Clínic de
Barcelona, Universitat de Barcelona, IDIBAPS, CERCA, Barcelona, Spain.
(11)Division of Neuropathology and Neurochemistry, Department of Neurology,
Medical University of Vienna, Vienna, Austria.
(12)Frontotemporal Degeneration Center, Department of Neurology, University of
Pennsylvania, Philadelphia, Pennsylvania, USA.
(13)Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
(14)Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands.
(15)London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry,
Psychology and Neuroscience, Kings College London, London, UK.
(16)University de Bordeaux, Institut des Maladies Neurodégénératives, CNRS UMR
5293, Bordeaux, France.
(17)Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
(18)University of Otago, Christchurch, and New Zealand Brain Research Institute,
Department Medicine, Christchurch, New Zealand.
(19)Department of Clinical Sciences, Division of Neurology, Lund University,
Lund, Sweden.
(20)Centro de Investigación Biomédica en Red sobre Enfermedades
Neurodegenerativas (CIBERNED), Hospital Clínic de Barcelona, Barcelona, Spain.
(21)Movement Disorders Unit, Neurology Service, Hospital Clínic de Barcelona.
Institute of Neuroscience, University of Barcelona, Barcelona, Spain.
(22)Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), Barcelona,
(23)Department of Clinical Neurosciences and Cambridge Centre for Parkinson-Plus,
Cambridge University, Cambridge, UK.

BACKGROUND: The Movement Disorder Society diagnostic criteria for progressive
supranuclear palsy introduced the diagnostic certainty level “suggestive of
progressive supranuclear palsy” for clinical conditions with subtle signs,
suggestive of the disease. This category aims at the early identification of
patients, in whom the diagnosis may be confirmed as the disease evolves.
OBJECTIVE: To assess the diagnostic performance of the defined clinical
conditions suggestive of progressive supranuclear palsy in an autopsy-confirmed
METHODS: Diagnostic performance of the criteria was analyzed based on
retrospective clinical data of 204 autopsy-confirmed patients with progressive
supranuclear palsy and 216 patients with other neurological diseases.
RESULTS: The conditions suggestive of progressive supranuclear palsy strongly
increased the sensitivity compared to the National Institute of Neurological
Disorders and Stroke and Society for Progressive Supranuclear Palsy criteria.
Within the first year after symptom onset, 40% of patients with definite
progressive supranuclear palsy fulfilled criteria for suggestive of progressive
supranuclear palsy. Two-thirds of patients suggestive of progressive supranuclear
palsy evolved into probable progressive supranuclear palsy after an average of
3.6 years. Application of the criteria for suggestive of progressive supranuclear
palsy reduced the average time to diagnosis from 3.8 to 2.2 years.
CONCLUSIONS: Clinical conditions suggestive of progressive supranuclear palsy
allow earlier identification of patients likely to evolve into clinically
possible or probable progressive supranuclear and to have underlying progressive
supranuclear palsy pathology. Further work needs to establish the specificity and
positive predictive value of this category in real-life clinical settings, and to
develop specific biomarkers that enhance their diagnostic accuracy in early
disease stages. © 2020 The Authors. Movement Disorders published by Wiley
Periodicals LLC on behalf of International Parkinson and Movement Disorder

© 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on
behalf of International Parkinson and Movement Disorder Society.


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