How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy

doi:10.1002/mds.27666

. 2019 Aug;34(8):1228-1232.

doi: 10.1002/mds.27666. Epub 2019 Mar 18.

How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy

Max-Joseph Grimm^{1

2}, Gesine Respondek^{1

2}, Maria Stamelou³, Thomas Arzberger^{2

4

5}, Leslie Ferguson⁶, Ellen Gelpi^{7

8}, Armin Giese⁴, Murray Grossman⁹, David J Irwin⁹, Alexander Pantelyat¹⁰, Alex Rajput⁶, Sigrun Roeber⁴, John C van Swieten¹¹, Claire Troakes¹², Angelo Antonini¹³, Kailash P Bhatia¹⁴, Carlo Colosimo¹⁵, Thilo van Eimeren¹⁶, Jan Kassubek¹⁷, Johannes Levin^{2

18}, Wassilios G Meissner^{19

20

21}, Christer Nilsson²², Wolfgang H Oertel²³, Ines Piot^{1

2}, Werner Poewe²⁴, Gregor K Wenning²⁴, Adam Boxer²⁵, Lawrence I Golbe²⁶, Keith A Josephs²⁷, Irene Litvan²⁸, Huw R Morris²⁹, Jennifer L Whitwell³⁰, Yaroslau Compta³¹, Jean-Christophe Corvol³², Anthony E Lang³³, James B Rowe³⁴, Günter U Höglinger^{1

2}; Movement Disorder Society-endorsed PSP Study Group

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 Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität, Munich, Germany.
⁵ Department of Psychiatry and Psychotherapy, University Hospital, LMU Munich, Munich, Germany.
⁶ Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
⁷ Neurological Tissue Bank and Neurology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, IDIBAPS, CERCA, Barcelona, Catalonia, Spain.
⁸ Institute of Neurology, Medical University of Vienna, Vienna, Austria.
⁹ Frontotemporal Degeneration Center, Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹⁰ Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
¹¹ Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands.
¹² London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry, Psychology and Neuroscience, Kings College London, London, UK.
¹³ Parkinson and Movement Disorders Unit, IRCCS Hospital San Camillo, Venice, and Department of Neurosciences, Padova University, Padova, Italy.
¹⁴ Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, UK.
¹⁵ Department of Neurology, Santa Maria University Hospital of Terni, Terni, Italy.
¹⁶ Departments of Nuclear Medicine and Neurology, University of Cologne, Cologne, Germany.
¹⁷ Department of Neurology, University of Ulm, Ulm, Germany.
¹⁸ Department of Neurology, Ludwig-Maximilians-Universität, Munich, Germany.
¹⁹ Univ. de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France.
²⁰ Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
²¹ Dept. Medicine, University of Otago, Christchurch, and New Zealand Brain Research Institute, Christchurch, New Zealand.
²² Department of Clinical Sciences, Division of Neurology, Lund University, Lund, Sweden.
²³ Department of Neurology, Philipps Universität, Marburg, Germany.
²⁴ Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.
²⁵ Memory and Aging Center, Department of Neurology, University of California, San Francisco, California, USA.
²⁶ Department of Neurology, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
²⁷ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
²⁸ Department of Neurology, University of California, San Diego, California, USA.
²⁹ Department of Clinical Neuroscience, UCL Institute of Neurology, Queen Square, London, UK.
³⁰ Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
³¹ Parkinson's Disease & Movement Disorders Unit, Hospital Clínic/IDIBAPS/CIBERNED/European Reference Network for Rare Neurological Diseases (ERN-RND)/Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia, Spain.
³² Sorbonne Université, INSERM, CNRS, Assistance Publique Hôpitaux de Paris, ICM, Hôpital Pitié-Salpêtrière, Department of Neurology, Paris, France.
³³ The Edmond J Safra Program in Parkinson's Disease and the Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, Toronto, Ontario, Canada.
³⁴ Department of Clinical Neurosciences and Cambridge Centre for Parkinson-Plus, Cambridge University, Cambridge, UK.

PMID: 30884545
PMCID: PMC6699888
DOI: 10.1002/mds.27666

How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy

Max-Joseph Grimm et al. Mov Disord. 2019 Aug.

. 2019 Aug;34(8):1228-1232.

doi: 10.1002/mds.27666. Epub 2019 Mar 18.

Authors

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 Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität, Munich, Germany.
⁵ Department of Psychiatry and Psychotherapy, University Hospital, LMU Munich, Munich, Germany.
⁶ Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
⁷ Neurological Tissue Bank and Neurology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, IDIBAPS, CERCA, Barcelona, Catalonia, Spain.
⁸ Institute of Neurology, Medical University of Vienna, Vienna, Austria.
⁹ Frontotemporal Degeneration Center, Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹⁰ Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA.
¹¹ Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands.
¹² London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry, Psychology and Neuroscience, Kings College London, London, UK.
¹³ Parkinson and Movement Disorders Unit, IRCCS Hospital San Camillo, Venice, and Department of Neurosciences, Padova University, Padova, Italy.
¹⁴ Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London, UK.
¹⁵ Department of Neurology, Santa Maria University Hospital of Terni, Terni, Italy.
¹⁶ Departments of Nuclear Medicine and Neurology, University of Cologne, Cologne, Germany.
¹⁷ Department of Neurology, University of Ulm, Ulm, Germany.
¹⁸ Department of Neurology, Ludwig-Maximilians-Universität, Munich, Germany.
¹⁹ Univ. de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France.
²⁰ Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
²¹ Dept. Medicine, University of Otago, Christchurch, and New Zealand Brain Research Institute, Christchurch, New Zealand.
²² Department of Clinical Sciences, Division of Neurology, Lund University, Lund, Sweden.
²³ Department of Neurology, Philipps Universität, Marburg, Germany.
²⁴ Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.
²⁵ Memory and Aging Center, Department of Neurology, University of California, San Francisco, California, USA.
²⁶ Department of Neurology, Rutgers Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
²⁷ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
²⁸ Department of Neurology, University of California, San Diego, California, USA.
²⁹ Department of Clinical Neuroscience, UCL Institute of Neurology, Queen Square, London, UK.
³⁰ Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
³¹ Parkinson's Disease & Movement Disorders Unit, Hospital Clínic/IDIBAPS/CIBERNED/European Reference Network for Rare Neurological Diseases (ERN-RND)/Institut de Neurociències, Universitat de Barcelona, Barcelona, Catalonia, Spain.
³² Sorbonne Université, INSERM, CNRS, Assistance Publique Hôpitaux de Paris, ICM, Hôpital Pitié-Salpêtrière, Department of Neurology, Paris, France.
³³ The Edmond J Safra Program in Parkinson's Disease and the Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital, Toronto, Ontario, Canada.
³⁴ Department of Clinical Neurosciences and Cambridge Centre for Parkinson-Plus, Cambridge University, Cambridge, UK.

PMID: 30884545
PMCID: PMC6699888
DOI: 10.1002/mds.27666

Abstract

Background: The Movement Disorder Society criteria for progressive supranuclear palsy define diagnostic allocations, stratified by certainty levels and clinical predominance types. We aimed to study the frequency of ambiguous multiple allocations and to develop rules to eliminate them.

Methods: We retrospectively collected standardized clinical data by chart review in a multicenter cohort of autopsy-confirmed patients with progressive supranuclear palsy, to classify them by diagnostic certainty level and predominance type and to identify multiple allocations.

Results: Comprehensive data were available from 195 patients. More than one diagnostic allocation occurred in 157 patients (80.5%). On average, 5.4 allocations were possible per patient. We developed four rules for Multiple Allocations eXtinction (MAX). They reduced the number of patients with multiple allocations to 22 (11.3%), and the allocations per patient to 1.1.

Conclusions: The proposed MAX rules help to standardize the application of the Movement Disorder Society criteria for progressive supranuclear palsy. © 2019 International Parkinson and Movement Disorder Society.

Keywords: autopsy; diversity; phenotype; progressive supranuclear palsy.

PubMed Disclaimer

Conflict of interest statement

Relevant conflicts of interest/financial disclosures: None.

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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. Kovacs GG. Neuropathology of tauopathies: principles and practice. Neuropathol Appl Neurobiol 2015;41:3–23. - PubMed
1. Respondek G, Kurz C, Arzberger T, et al., for the Movement Disorder Society-endorsed PSP Study Group. Which ante-mortem features predict progressive supranuclear palsy? Mov Disord 2017;32(7): 995–1005. - PMC - PubMed
1. Höglinger GU, Respondek G, Stamelou M, et al., for the Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy — the Movement Disorder Society Criteria. Mov Disord 2017;32(6):853–864. - PMC - PubMed
1. Ali F, Martin P, Botha H, et al. Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy. Move Disord 2019; doi: 10.1002/mds.27619. - DOI - PMC - PubMed

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[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

[2] 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

[3] Kovacs GG. Neuropathology of tauopathies: principles and practice. Neuropathol Appl Neurobiol 2015;41:3–23. - PubMed

[4] Kovacs GG. Neuropathology of tauopathies: principles and practice. Neuropathol Appl Neurobiol 2015;41:3–23. - PubMed

[5] Respondek G, Kurz C, Arzberger T, et al., for the Movement Disorder Society-endorsed PSP Study Group. Which ante-mortem features predict progressive supranuclear palsy? Mov Disord 2017;32(7): 995–1005. - PMC - PubMed

[6] Respondek G, Kurz C, Arzberger T, et al., for the Movement Disorder Society-endorsed PSP Study Group. Which ante-mortem features predict progressive supranuclear palsy? Mov Disord 2017;32(7): 995–1005. - PMC - PubMed

[7] Höglinger GU, Respondek G, Stamelou M, et al., for the Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy — the Movement Disorder Society Criteria. Mov Disord 2017;32(6):853–864. - PMC - PubMed

[8] Höglinger GU, Respondek G, Stamelou M, et al., for the Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy — the Movement Disorder Society Criteria. Mov Disord 2017;32(6):853–864. - PMC - PubMed

[9] Ali F, Martin P, Botha H, et al. Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy. Move Disord 2019; doi: 10.1002/mds.27619. - DOI - PMC - PubMed

[10] Ali F, Martin P, Botha H, et al. Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy. Move Disord 2019; doi: 10.1002/mds.27619. - DOI - PMC - PubMed

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How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy

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How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy

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