A Modified Progressive Supranuclear Palsy Rating Scale
Mov Disord. 2021-01-29; 36(5): 1203-1215
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Grötsch MT(1)(2), Respondek G(1)(2)(3), Colosimo C(4), Compta Y(5), Corvol JC(6), Ferreira J(7), Huber MK(3), Klietz M(3), Krey LFM(3), Levin J(2)(8),
Jecmenica-Lukic M(9), Macías-García D(10), Meissner WG(11)(12)(13), Mir P(10)(14), Morris H(15), Nilsson C(16), Rowe JB(17), Seppi K(18), Stamelou
M(19)(20)(21), van Swieten JC(22), Wenning G(18), Del Ser T(23), Golbe LI(24), Höglinger GU(1)(2)(3); Describe PSP Study Group, the ProPSP Study Group, and the Movement Disorder Society-Endorsed PSP Study Group.
(1)Department of Neurology, Technische Universität München, Munich, Germany.
(2)German Center for Neurodegenerative Diseases, Munich, Germany.
(3)Department of Neurology, Hanover Medical School, Hanover, Germany.
(4)Department of Neurology, Santa Maria University Hospital of Terni, Terni,
(5)Parkinson’s Disease and Movement Disorders Unit, Hospital
Clínic/IDIBAPS/CIBERNED/(CB06/05/0018-ISCIII)/European Reference Network for Rare
Neurological Diseases (ERN-RND)/Institut de Neurociències, Universitat de
Barcelona, Catalonia, Spain.
(6)Département des Maladies du Système Nerveux, Sorbonne Universités, UPMC Univ
Paris 06 INSERM UMRS_1127, CIC_1422; CNRS UMR_7225; AP-HP; and ICM, Hôpital
Pitié-Salpêtrière, Paris, France.
(7)Faculdade de Medicina de Lisboa, Lisbon, Portugal.
(8)Department of Neurology, Ludwig-Maximilians-Universität, Munich, Germany.
(9)Movement Disorders Department, Clinic for Neurology, Belgrade, Serbia.
(10)Unidad de Trastornos del Movimiento, Servicio de Neurología y Neurofisiología
Clínica, Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del
Rocío/CSIC/Universidad de Sevilla, Seville, Spain.
(11)University de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293,
(12)Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France.
(13)Department of Medicine, University of Otago, Christchurch, and New Zealand
Brain Research Institute, Christchurch, New Zealand.
(14)Centro de Investigación Biomédica en Red sobre Enfermedades
Neurodegenerativas (CIBERNED), Madrid, Spain.
(15)Department of Clinical Neuroscience, UCL Institute of Neurology, London,
(16)Department of Clinical Sciences, Division of Neurology, Lund University,
(17)Department of Clinical Neurosciences and Cambridge Centre for Parkinson-Plus,
Cambridge University, Cambridge, United Kingdom.
(18)Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.
(19)Parkinson’s Disease and Movement Disorders Department, HYGEIA Hospital,
(20)Philipps University Marburg, Marburg, Germany.
(21)School of Medicine, European University of Cyprus, Nicosia, Cyprus.
(22)Department of Neurology, Erasmus Medical Centre, Rotterdam, the Netherlands.
(23)Alzheimer’s Disease Investigation Research Unit, CIEN Foundation, Carlos III
Institute of Health, Noscira SA, Madrid, Spain.
(24)Department of Neurology, Rutgers Robert Wood Johnson Medical School, New
Brunswick, New Jersey, USA.
BACKGROUND: The Progressive Supranuclear Palsy Rating Scale is a prospectively
validated physician-rated measure of disease severity for progressive
supranuclear palsy. We hypothesized that, according to experts’ opinion,
individual scores of items would differ in relevance for patients’ quality of
life, functionality in daily living, and mortality. Thus, changes in the score
may not equate to clinically meaningful changes in the patient’s status.
OBJECTIVE: The aim of this work was to establish a condensed modified version of
the scale focusing on meaningful disease milestones.
METHODS: Sixteen movement disorders experts evaluated each scale item for its
capacity to capture disease milestones (0 = no, 1 = moderate, 2 = severe
milestone). Items not capturing severe milestones were eliminated. Remaining
items were recalibrated in proportion to milestone severity by collapsing across
response categories that yielded identical milestone severity grades. Items with
low sensitivity to change were eliminated, based on power calculations using
longitudinal 12-month follow-up data from 86 patients with possible or probable
progressive supranuclear palsy.
RESULTS: The modified scale retained 14 items (yielding 0-2 points each). The
items were rated as functionally relevant to disease milestones with comparable
severity. The modified scale was sensitive to change over 6 and 12 months and of
similar power for clinical trials of disease-modifying therapy as the original
scale (achieving 80% power for two-sample t test to detect a 50% slowing with
n = 41 and 25% slowing with n = 159 at 12 months).
CONCLUSIONS: The modified Progressive Supranuclear Palsy Rating Scale may serve
as a clinimetrically sound scale to monitor disease progression in clinical
trials and routine. © 2021 International Parkinson and Movement Disorder Society.
© 2021 International Parkinson and Movement Disorder Society.