. 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

Günter U Höglinger^{1

2}, Gesine Respondek^{1

2}, Maria Stamelou³, Carolin Kurz⁴, Keith A Josephs⁵, Anthony E Lang⁶, Brit Mollenhauer⁷, Ulrich Müller⁸, Christer Nilsson⁹, Jennifer L Whitwell¹⁰, Thomas Arzberger^{2

4

11}, Elisabet Englund¹², Ellen Gelpi¹³, Armin Giese¹¹, David J Irwin¹⁴, Wassilios G Meissner^{15

16

17}, Alexander Pantelyat¹⁸, Alex Rajput¹⁹, John C van Swieten²⁰, Claire Troakes²¹, Angelo Antonini²², Kailash P Bhatia²³, Yvette Bordelon²⁴, Yaroslau Compta²⁵, Jean-Christophe Corvol²⁶, Carlo Colosimo²⁷, Dennis W Dickson²⁸, Richard Dodel²⁹, Leslie Ferguson¹⁹, Murray Grossman¹⁴, Jan Kassubek³⁰, Florian Krismer³¹, Johannes Levin^{2

32}, Stefan Lorenzl^{33

34

35}, Huw R Morris³⁶, Peter Nestor³⁷, Wolfgang H Oertel³⁸, Werner Poewe³¹, Gil Rabinovici³⁹, James B Rowe⁴⁰, Gerard D Schellenberg⁴¹, Klaus Seppi³¹, Thilo van Eimeren⁴², Gregor K Wenning³¹, Adam L Boxer³⁹, Lawrence I Golbe⁴³, Irene Litvan⁴⁴; Movement Disorder Society-endorsed PSP Study Group¹

Collaborators, Affiliations

Collaborators

Movement Disorder Society-endorsed PSP Study Group:
Adam L Boxer, Alex Rajput, Alexander Pantelyat, Angelo Antonini, Anthony E Lang, Armin Giese, Brit Mollenhauer, Carlo Colosimo, Caroline Kurz, Christer Nilsson, Claire Troakes, David J Irwin, Dennis W Dickson, Ellen Gelpi, Florian Krismer, Gerard D Schellenberg, Gesine Respondek, Gil Rabinovici, Gregor K Wenning, Günter U Höglinger, Huw R Morris, Irene Litvan, James B Rowe, Jan Kassubek, Jean-Christophe Corvol, Jennifer L Whitwell, Johannes Levin, John van Swieten, Kailash P Bhatia, Keith A Josephs, Klaus Seppi, Lawrence I Golbe, Maria Stamelou, Murray Grossman, Peter Nestor, Richard Dodel, Stefan Lorenzl, Thilo van Eimeren, Thomas Arzberger, Ulrich Müller, Wassilios G Meissner, Werner Poewe, Wolfgang H Oertel, Yaroslau Compta, Yvette Bordelon

Affiliations

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, Sweden.
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, USA.
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, Italy.
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 Kingdom.
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, USA.

PMID: 28467028
PMCID: PMC5516529
DOI: 10.1002/mds.26987

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

Günter U Höglinger et al. Mov Disord. 2017 Jun.

. 2017 Jun;32(6):853-864.

doi: 10.1002/mds.26987. Epub 2017 May 3.

Authors

Collaborators

Movement Disorder Society-endorsed PSP Study Group:
Adam L Boxer, Alex Rajput, Alexander Pantelyat, Angelo Antonini, Anthony E Lang, Armin Giese, Brit Mollenhauer, Carlo Colosimo, Caroline Kurz, Christer Nilsson, Claire Troakes, David J Irwin, Dennis W Dickson, Ellen Gelpi, Florian Krismer, Gerard D Schellenberg, Gesine Respondek, Gil Rabinovici, Gregor K Wenning, Günter U Höglinger, Huw R Morris, Irene Litvan, James B Rowe, Jan Kassubek, Jean-Christophe Corvol, Jennifer L Whitwell, Johannes Levin, John van Swieten, Kailash P Bhatia, Keith A Josephs, Klaus Seppi, Lawrence I Golbe, Maria Stamelou, Murray Grossman, Peter Nestor, Richard Dodel, Stefan Lorenzl, Thilo van Eimeren, Thomas Arzberger, Ulrich Müller, Wassilios G Meissner, Werner Poewe, Wolfgang H Oertel, Yaroslau Compta, Yvette Bordelon

Affiliations

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, Sweden.
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, USA.
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, Italy.
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 Kingdom.
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, USA.

PMID: 28467028
PMCID: PMC5516529
DOI: 10.1002/mds.26987

Abstract

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 rounds.

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 features.

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.

Keywords: clinical diagnostic criteria; consensus-based; evidence-based; progressive supranuclear palsy.

PubMed Disclaimer

Conflict of interest statement

Relevant conflicts of interest/financial disclosures: Nothing to report. Full financial disclosures and author roles may be found in the online version of this article.

References

1. Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol. 1964;10:333–359. - PubMed
1. Litvan I, Hauw JJ, Bartko JJ, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supra-nuclear palsy and related disorders. J Neuropathol Exp Neurol. 1996;55:97–105. - PubMed
1. Dickson DW. Neuropathologic differentiation of progressive supranuclear palsy and corticobasal degeneration. J Neurol. 1999;246(Suppl 2):II6–II15. - PubMed
1. Kovacs GG. Neuropathology of tauopathies: principles and practice. Neuropathol Appl Neurobiol. 2015;41:3–23. - PubMed
1. Litvan I, Agid Y, Calne D, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996;47:1–9. - PubMed

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

Collaborators

Affiliations

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

Authors

Collaborators

Affiliations

Abstract

Conflict of interest statement

References

Publication types

MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical

Miscellaneous