Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria

Günter U. Höglinger, Gesine Respondek, Maria Stamelou, Carolin Kurz, Keith A. Josephs, Anthony E. Lang, Brit Mollenhauer, Ulrich Müller, Christer Nilsson, Jennifer L. Whitwell, Thomas Arzberger, Elisabet Englund, Ellen Gelpi, Armin Giese, David J. Irwin, Wassilios G. Meissner, Alexander Pantelyat, Alex Rajput, John C. van Swieten, Claire Troakes, Angelo Antonini, Kailash P. Bhatia, Yvette Bordelon, Yaroslau Compta, Jean-Christophe Corvol, Carlo Colosimo, Dennis W. Dickson, Richard Dodel, Leslie Ferguson, Murray Grossman, Jan Kassubek, Florian Krismer, Johannes Levin, Stefan Lorenzl, Huw R. Morris, Peter Nestor, Wolfgang H. Oertel, Werner Poewe, Gil Rabinovici, James B. Rowe, Gerard D. Schellenberg, Klaus Seppi, Thilo van Eimeren, Gregor K. Wenning, Adam L. Boxer, Lawrence I. Golbe, Irene Litvan,
Mov Disord.. 2017-05-03; 32(6): 853-864
DOI: 10.1002/mds.26987

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1. Mov Disord. 2017 Jun;32(6):853-864. doi: 10.1002/mds.26987. Epub 2017 May 3.

Clinical diagnosis of progressive supranuclear palsy: The movement disorder
society criteria.

Höglinger GU(1)(2), Respondek G(1)(2), Stamelou M(3), Kurz C(4), Josephs KA(5),
Lang AE(6), Mollenhauer B(7), Müller U(8), Nilsson C(9), Whitwell JL(10),
Arzberger T(2)(4)(11), Englund E(12), Gelpi E(13), Giese A(11), Irwin DJ(14),
Meissner WG(15)(16)(17), Pantelyat A(18), Rajput A(19), van Swieten JC(20),
Troakes C(21), Antonini A(22), Bhatia KP(23), Bordelon Y(24), Compta Y(25),
Corvol JC(26), Colosimo C(27), Dickson DW(28), Dodel R(29), Ferguson L(19),
Grossman M(14), Kassubek J(30), Krismer F(31), Levin J(2)(32), Lorenzl
S(33)(34)(35), Morris HR(36), Nestor P(37), Oertel WH(38), Poewe W(31),
Rabinovici G(39), Rowe JB(40), Schellenberg GD(41), Seppi K(31), van Eimeren
T(42), Wenning GK(31), Boxer AL(39), Golbe LI(43), Litvan I(44); Movement
Disorder Society-endorsed PSP Study Group(1).

Collaborators: Boxer AL, Rajput A, Pantelyat A, Antonini A, Lang AE, Giese A,
Mollenhauer B, Colosimo C, Kurz C, Nilsson C, Troakes C, Irwin DJ, Dickson DW,
Gelpi E, Krismer F, Schellenberg GD, Respondek G, Rabinovici G, Wenning GK,
Höglinger GU, Morris HR, Litvan I, Rowe JB, Kassubek J, Corvol JC, Whitwell JL,
Levin J, van Swieten J, Bhatia KP, Josephs KA, Seppi K, Golbe LI, Stamelou M,
Grossman M, Nestor P, Dodel R, Lorenzl S, van Eimeren T, Arzberger T, Müller U,
Meissner WG, Poewe W, Oertel WH, Compta Y, Bordelon Y.

Author information:
(1)Department of Neurology, Technische Universität München, Munich, Germany.
(2)German Center for Neurodegenerative Diseases (DZNE), Munich, Germany.
(3)Second Department of Neurology, Attikon University Hospital, University of
Athens, Athens, Greece.
(4)Department of Psychiatry, Ludwig-Maximilians-Universität, Munich, Germany.
(5)Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
(6)Morton and Gloria Shulman Movement Disorders Clinic and the Edmond J. Safra
Program in Parkinson’s Disease, Toronto Western Hospital, Toronto, Canada.
(7)Paracelsus-Elena Klinik, Kassel, Germany, and University Medical Center
Göttingen, Institute of Neuropathology, Göttingen, Germany.
(8)Institute of Human Genetics, Giessen, Germany.
(9)Department of Clinical Sciences, Division of Neurology, Lund University, Lund,
(10)Department of Radiology, Mayo Clinic, Rochester, Minnesoya, USA.
(11)Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität,
Munich, Germany.
(12)Department of Clinical Sciences, Division of Oncology and Pathology, Lund
University, Lund, Sweden.
(13)Neurological Tissue Bank of the Biobank – Hospital Clínic de Barcelona,
Universitat de Barcelona, IDIBAPS, Barcelona, Spain.
(14)Frontotemporal Degeneration Center, Department of Neurology, University of
Pennsylvania, Philadelphia, Pennsylvania, USA.
(15)Université de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293,
Bordeaux, France.
(16)CNRS, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France.
(17)Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
(18)Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
(19)Division of Neurology, Royal University Hospital, University of Saskatchewan,
Saskatoon, SK, Canada.
(20)Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands.
(21)London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry,
Psychology and Neuroscience, Kings College London, London, United Kingdom.
(22)Parkinson and Movement Disorders Unit, IRCCS Hospital San Camillo, Venice,
and Department of Neurosciences, Padova University, Padova, Italy.
(23)Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute
of Neurology, Queen Square, London, United Kingdom.
(24)Department of Neurology, University of California, Los Angeles, California,
(25)Parkinson’s Disease & Movement Disorders Unit, Neurology Service, Hospital
Clinic/IDIBAPS/University of Barcelona, Barcelona, Catalonia, Spain.
(26)Sorbonne Universités, UPMC Univ Paris 06; and INSERM UMRS_1127, CIC_1422; and
CNRS UMR_7225; and AP-HP; and ICM, Hôpital Pitié-Salpêtrière, Département des
maladies du système nerveux, Paris, France.
(27)Department of Neurology, Santa Maria University Hospital of Terni, Terni,
(28)Mayo Clinic, Jacksonville, Florida, USA.
(29)Department of Geriatric Medicine, University Hospital Essen, Essen, Germany.
(30)Department of Neurology, University of Ulm, Ulm, Germany.
(31)Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.
(32)Department of Neurology, Ludwig-Maximilians-Universität, Munich, Germany.
(33)Institute of Nursing Science and Practice, Paracelsus Medical University,
Salzburg, Austria.
(34)Department of Neurology, Hospital Agatharied, Agatharied, Germany.
(35)Department of Palliative Medicine, Munich University Hospital, LMU Munich,
Munich, Germany.
(36)Department of Clinical Neuroscience, UCL Institute of Neurology, London,
United Kingdom.
(37)German Center for Neurodegenerative Diseases (DZNE), Magdeburg, Germany.
(38)Department of Neurology, Philipps Universität, Marburg, Germany.
(39)Memory and Aging Center, Department of Neurology, University of California,
San Francisco, California, USA.
(40)Department of Clinical Neurosciences, Cambridge University, Cambridge, United
(41)Department of Pathology and Laboratory Medicine, Perelman School of Medicine,
University of Pennsylvania, Philadelphia, Pennsylvania, USA.
(42)Departments of Nuclear Medicine and Neurology, University of Cologne,
Cologne, Germany.
(43)Department of Neurology, Rutgers Robert Wood Johnson Medical School, New
Brunswick, New Jersey, USA.
(44)Department of Neurology, University of California, San Diego, California,

BACKGROUND: PSP is a neuropathologically defined disease entity. Clinical
diagnostic criteria, published in 1996 by the National Institute of Neurological
Disorders and Stroke/Society for PSP, have excellent specificity, but their
sensitivity is limited for variant PSP syndromes with presentations other than
Richardson’s syndrome.
OBJECTIVE: We aimed to provide an evidence- and consensus-based revision of the
clinical diagnostic criteria for PSP.
METHODS: We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for
articles published in English since 1996, using postmortem diagnosis or highly
specific clinical criteria as the diagnostic standard. Second, we generated
retrospective standardized clinical data from patients with autopsy-confirmed PSP
and control diseases. On this basis, diagnostic criteria were drafted, optimized
in two modified Delphi evaluations, submitted to structured discussions with
consensus procedures during a 2-day meeting, and refined in three further Delphi
RESULTS: Defined clinical, imaging, laboratory, and genetic findings serve as
mandatory basic features, mandatory exclusion criteria, or context-dependent
exclusion criteria. We identified four functional domains (ocular motor
dysfunction, postural instability, akinesia, and cognitive dysfunction) as
clinical predictors of PSP. Within each of these domains, we propose three
clinical features that contribute different levels of diagnostic certainty.
Specific combinations of these features define the diagnostic criteria,
stratified by three degrees of diagnostic certainty (probable PSP, possible PSP,
and suggestive of PSP). Clinical clues and imaging findings represent supportive
CONCLUSIONS: Here, we present new criteria aimed to optimize early, sensitive,
and specific clinical diagnosis of PSP on the basis of currently available
evidence. © 2017 International Parkinson and Movement Disorder Society.

© 2017 International Parkinson and Movement Disorder Society.

DOI: 10.1002/mds.26987
PMCID: PMC5516529
PMID: 28467028 [Indexed for MEDLINE]

Auteurs Bordeaux Neurocampus