{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T22:20:07Z","timestamp":1792794007285,"version":"4.5.0"},"reference-count":49,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2023,3,28]],"date-time":"2023-03-28T00:00:00Z","timestamp":1679961600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"PROSPECT"},{"DOI":"10.13039\/100013128","name":"MSA Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013128","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["103838"],"award-info":[{"award-number":["103838"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["220258"],"award-info":[{"award-number":["220258"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"NIHR","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Cambridge Brain Bank","award":["BRC-1215-20014"],"award-info":[{"award-number":["BRC-1215-20014"]}]},{"DOI":"10.13039\/501100000276","name":"Department of Health and Social Care","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000276","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008721","name":"UCLH","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008721","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100022596","name":"Reta Lila Weston Institute for Neurological Studies","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100022596","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"MRC","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011085","name":"Biomedical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011085","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000320","name":"Alzheimer\u2019s Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000320","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002283","name":"Alzheimer\u2019s Research UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002283","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007155","name":"Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007155","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Association of British Neurologists Clinical Research","id":[{"id":"https:\/\/ror.org\/00n9sg246","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000627","name":"Guarantors of Brain","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000627","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000320","name":"a Fellowship award from the Alzheimer\u2019s Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000320","id-type":"DOI","asserted-by":"publisher"}]},{"name":"UK Dementia Research Institute which receives its funding from DRI Ltd","id":[{"id":"https:\/\/ror.org\/02wedp412","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000690","name":"UK Medical Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000690","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000320","name":"Alzheimer\u2019s Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000320","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002283","name":"Alzheimer\u2019s Research UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002283","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013790","name":"Miriam Marks Brain Research UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013790","id-type":"DOI","asserted-by":"publisher"}]},{"name":"MRC Clinician Scientist Fellowship"},{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000781","name":"European Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000781","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002565","name":"Alzheimer Drug Discovery Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002565","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002565","name":"ADDF","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002565","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000957","name":"Alzheimer's Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000957","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007436","name":"Erling-Persson Family Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007436","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010815","name":"Stiftelsen f\u00f6r Gamla Tj\u00e4narinnor","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010815","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003792","name":"Hj\u00e4rnfonden","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003792","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007601","name":"Horizon 2020","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007601","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Manchester Brain Bank","id":[{"id":"https:\/\/ror.org\/05njkjr15","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000320","name":"Alzheimer\u2019s Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000320","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002283","name":"Alzheimer\u2019s Research UK","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002283","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,8,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The advent of clinical trials of disease-modifying agents for neurodegenerative disease highlights the need for evidence-based end point selection. Here we report the longitudinal PROSPECT-M-UK study of progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), multiple system atrophy (MSA) and related disorders, to compare candidate clinical trial end points.<\/jats:p>\n                  <jats:p>In this multicentre UK study, participants were assessed with serial questionnaires, motor examination, neuropsychiatric and MRI assessments at baseline, 6 and 12 months. Participants were classified by diagnosis at baseline and study end, into Richardson syndrome, PSP-subcortical (PSP-parkinsonism and progressive gait freezing subtypes), PSP-cortical (PSP-frontal, PSP-speech and language and PSP-CBS subtypes), MSA-parkinsonism, MSA-cerebellar, CBS with and without evidence of Alzheimer\u2019s disease pathology and indeterminate syndromes. We calculated annual rate of change, with linear mixed modelling and sample sizes for clinical trials of disease-modifying agents, according to group and assessment type.<\/jats:p>\n                  <jats:p>Two hundred forty-three people were recruited [117 PSP, 68 CBS, 42 MSA and 16 indeterminate; 138 (56.8%) male; age at recruitment 68.7 \u00b1 8.61 years]. One hundred and fifty-nine completed the 6-month assessment (82 PSP, 27 CBS, 40 MSA and 10 indeterminate) and 153 completed the 12-month assessment (80 PSP, 29 CBS, 35 MSA and nine indeterminate). Questionnaire, motor examination, neuropsychiatric and neuroimaging measures declined in all groups, with differences in longitudinal change between groups. Neuroimaging metrics would enable lower sample sizes to achieve equivalent power for clinical trials than cognitive and functional measures, often achieving N &amp;lt; 100 required for 1-year two-arm trials (with 80% power to detect 50% slowing). However, optimal outcome measures were disease-specific.<\/jats:p>\n                  <jats:p>In conclusion, phenotypic variance within PSP, CBS and MSA is a major challenge to clinical trial design. Our findings provide an evidence base for selection of clinical trial end points, from potential functional, cognitive, clinical or neuroimaging measures of disease progression.<\/jats:p>","DOI":"10.1093\/brain\/awad105","type":"journal-article","created":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T21:25:13Z","timestamp":1679606713000},"page":"3232-3242","source":"Crossref","is-referenced-by-count":46,"title":["Progression of atypical parkinsonian syndromes: PROSPECT-M-UK study implications for clinical trials"],"prefix":"10.1093","volume":"146","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2168-2242","authenticated-orcid":false,"given":"Duncan","family":"Street","sequence":"first","affiliation":[{"name":"University of Cambridge Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust , Cambridge, CB2 OQQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6844-882X","authenticated-orcid":false,"given":"Edwin","family":"Jabbari","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Movement Disorders Centre, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alyssa","family":"Costantini","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Movement Disorders Centre, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P Simon","family":"Jones","sequence":"additional","affiliation":[{"name":"University of Cambridge Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust , Cambridge, CB2 OQQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Negin","family":"Holland","sequence":"additional","affiliation":[{"name":"University of Cambridge Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust , Cambridge, CB2 OQQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1063-6937","authenticated-orcid":false,"given":"Timothy","family":"Rittman","sequence":"additional","affiliation":[{"name":"University of Cambridge Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust , Cambridge, CB2 OQQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marte T","family":"Jensen","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Movement Disorders Centre, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7797-0756","authenticated-orcid":false,"given":"Viorica","family":"Chelban","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Neurobiology and Medical Genetics Laboratory, \u2018Nicolae Testemitanu\u2019 State University of Medicine and Pharmacy , Chisinau 2004 , Republic of Moldova"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yen Y","family":"Goh","sequence":"additional","affiliation":[{"name":"Department of Neuromuscular Diseases, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tong","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda J","family":"Heslegrave","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"UK Dementia Research Institute, University College London , London, W1T 7NF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federico","family":"Roncaroli","sequence":"additional","affiliation":[{"name":"Geoffrey Jefferson Brain Research Centre, Division of Neuroscience, Faculty of Biology, Medicine and Health, University of Manchester , Manchester, M6 8HD , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes C","family":"Klein","sequence":"additional","affiliation":[{"name":"Wellcome Centre for Integrative Neuroimaging, Oxford Centre for Functional MRI of the Brain, Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford, OX3 9DU , UK"},{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford, OX3 9DU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olaf","family":"Ansorge","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford, OX3 9DU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kieren S J","family":"Allinson","sequence":"additional","affiliation":[{"name":"University of Cambridge Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust , Cambridge, CB2 OQQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zane","family":"Jaunmuktane","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Queen Square Brain Bank for Neurological Disorders, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Reta Lila Weston Institute, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamas","family":"Revesz","sequence":"additional","affiliation":[{"name":"Queen Square Brain Bank for Neurological Disorders, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Reta Lila Weston Institute, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6195-6995","authenticated-orcid":false,"given":"Thomas T","family":"Warner","sequence":"additional","affiliation":[{"name":"Queen Square Brain Bank for Neurological Disorders, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Reta Lila Weston Institute, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2476-4385","authenticated-orcid":false,"given":"Andrew J","family":"Lees","sequence":"additional","affiliation":[{"name":"Queen Square Brain Bank for Neurological Disorders, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Reta Lila Weston Institute, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrik","family":"Zetterberg","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"UK Dementia Research Institute, University College London , London, W1T 7NF , UK"},{"name":"Clinical Neurochemistry Laboratory, Sahlgrenska University Hospital , 431 30 M\u00f6lndal , Sweden"},{"name":"Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, The Salhgrenska Academy at the University of Gothenburg , 413 45 Goteborg , Sweden"},{"name":"Hong Kong Center for Neurodegenerative Diseases, Hong Kong Science Park , Shatin, N.T., Hong Kong , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucy L","family":"Russell","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1814-5024","authenticated-orcid":false,"given":"Martina","family":"Bocchetta","sequence":"additional","affiliation":[{"name":"Centre for Cognitive and Clinical Neuroscience, Division of Psychology, Department of Life Sciences, College of Health, Medicine and Life Sciences, Brunel University London , London, UB8 3PH , UK"},{"name":"Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D","family":"Rohrer","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David J","family":"Burn","sequence":"additional","affiliation":[{"name":"Faculty of Medical Sciences, Newcastle University , Newcastle, NE2 4HH , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicola","family":"Pavese","sequence":"additional","affiliation":[{"name":"Clinical Ageing Research Unit, Newcastle University , Newcastle, NE4 5PL , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8071-6062","authenticated-orcid":false,"given":"Alexander","family":"Gerhard","sequence":"additional","affiliation":[{"name":"Division of Neuroscience, Wolfson Molecular Imaging Centre, University of Manchester , Manchester, N20 3LJ , UK"},{"name":"Departments of Geriatric Medicine and Nuclear Medicine, Center for Translational Neuro- and Behavioral Sciences, University Medicine Essen , 45356 Essen , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"Kobylecki","sequence":"additional","affiliation":[{"name":"Division of Neuroscience, Wolfson Molecular Imaging Centre, University of Manchester , Manchester, N20 3LJ , UK"},{"name":"Department of Neurology, Manchester Academic Health Science Centre, Northern Care Alliance NHS Foundation Trust , Salford, M13 9NQ , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P Nigel","family":"Leigh","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Brighton and Sussex Medical School , Brighton, BN1 9PX , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alistair","family":"Church","sequence":"additional","affiliation":[{"name":"Department of Neurology, Royal Gwent Hospital , Newport, NP20 2UB , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele T M","family":"Hu","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford, OX3 9DU , UK"},{"name":"Department of Physiology, Anatomy and Genetics, Oxford Parkinson\u2019s Disease Centre, University of Oxford , Oxford, OX1 3QU , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2866-7777","authenticated-orcid":false,"given":"Henry","family":"Houlden","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Movement Disorders Centre, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Department of Neuromuscular Diseases, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5473-3774","authenticated-orcid":false,"given":"Huw","family":"Morris","sequence":"additional","affiliation":[{"name":"Department of Clinical and Movement Neurosciences, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"},{"name":"Movement Disorders Centre, University College London, Queen Square Institute of Neurology , London, WC1N 3BG , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James B","family":"Rowe","sequence":"additional","affiliation":[{"name":"University of Cambridge Department of Clinical Neurosciences and Cambridge University Hospitals NHS Trust , Cambridge, CB2 OQQ , UK"},{"name":"Medical Research Council Cognition and Brain Sciences Unit, University of Cambridge , Cambridge, CB2 7EF , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,3,28]]},"reference":[{"key":"2023080120203114100_awad105-B1","doi-asserted-by":"crossref","first-page":"1771","DOI":"10.1016\/S0140-6736(99)04137-9","article-title":"Prevalence of progressive supranuclear palsy and multiple system atrophy: A cross-sectional study","volume":"354","author":"Schrag","year":"1999","journal-title":"Lancet"},{"key":"2023080120203114100_awad105-B2","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/brain\/124.7.1438","article-title":"The prevalence of progressive supranuclear palsy (steele-richardson-olszewski syndrome) in the UK","volume":"124","author":"Nath","year":"2001","journal-title":"Brain"},{"key":"2023080120203114100_awad105-B3","doi-asserted-by":"crossref","first-page":"1736","DOI":"10.1212\/WNL.0000000000002638","article-title":"Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes","volume":"86","author":"Coyle-Gilchrist","year":"2016","journal-title":"Neurology"},{"key":"2023080120203114100_awad105-B4","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1002\/mds.26987","article-title":"Clinical diagnosis of progressive supranuclear palsy\u2014The movement disorder society criteria","volume":"32","author":"H\u00f6glinger","year":"2017","journal-title":"Mov Disord"},{"key":"2023080120203114100_awad105-B5","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1212\/WNL.0b013e31827f0fd1","article-title":"Criteria for the diagnosis of corticobasal degeneration","volume":"80","author":"Armstrong","year":"2013","journal-title":"Neurology"},{"key":"2023080120203114100_awad105-B6","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1212\/01.wnl.0000324625.00404.15","article-title":"Second consensus statement on the diagnosis of multiple system atrophy","volume":"71","author":"Gilman","year":"2008","journal-title":"Neurology"},{"key":"2023080120203114100_awad105-B7","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1007\/s100720100021","article-title":"Comparison of natural histories of progressive supranuclear palsy and multiple system atrophy","volume":"22","author":"Testa","year":"2001","journal-title":"Neurol Sci"},{"key":"2023080120203114100_awad105-B8","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1001\/jamaneurol.2019.4347","article-title":"Diagnosis across the Spectrum of progressive supranuclear palsy and corticobasal syndrome","volume":"77","author":"Jabbari","year":"2020","journal-title":"JAMA Neurol"},{"key":"2023080120203114100_awad105-B9","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1093\/brain\/awaa097","article-title":"Redefining the multidimensional clinical phenotypes of frontotemporal lobar degeneration syndromes","volume":"143","author":"Murley","year":"2020","journal-title":"Brain"},{"key":"2023080120203114100_awad105-B10","doi-asserted-by":"crossref","first-page":"fcab206","DOI":"10.1093\/braincomms\/fcab206","article-title":"Clinical progression of progressive supranuclear palsy: Impact of trials bias and phenotype variants","volume":"3","author":"Street","year":"2021","journal-title":"Brain Commun"},{"key":"2023080120203114100_awad105-B11","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1002\/mdc3.13010","article-title":"A convenient prognostic tool and staging system for progressive supranuclear palsy","volume":"7","author":"Golbe","year":"2020","journal-title":"Mov Disord Clin Pract"},{"key":"2023080120203114100_awad105-B12","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.parkreldis.2021.01.015","article-title":"Association of PSP phenotypes with survival: a brain-bank study","volume":"84","author":"Guasp","year":"2021","journal-title":"Park Relat Disord"},{"key":"2023080120203114100_awad105-B13","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1093\/brain\/awn291","article-title":"Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS Study","volume":"132","author":"Bensimon","year":"2009","journal-title":"Brain"},{"key":"2023080120203114100_awad105-B14","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1002\/ana.22424","article-title":"Clinicopathological correlations in corticobasal degeneration","volume":"70","author":"Lee","year":"2011","journal-title":"Ann Neurol"},{"key":"2023080120203114100_awad105-B15","doi-asserted-by":"crossref","first-page":"2016","DOI":"10.1212\/WNL.0000000000003305","article-title":"Progression of brain atrophy in PSP and CBS over 6 months and 1 year","volume":"87","author":"Dutt","year":"2016","journal-title":"Neurology"},{"key":"2023080120203114100_awad105-B16","doi-asserted-by":"crossref","first-page":"104813","DOI":"10.1016\/j.nbd.2020.104813","article-title":"Disease progression and prognostic factors in multiple system atrophy: A prospective cohort study","volume":"139","author":"Foubert-Samier","year":"2020","journal-title":"Neurobiol Dis"},{"key":"2023080120203114100_awad105-B17","doi-asserted-by":"crossref","first-page":"980","DOI":"10.1016\/j.parkreldis.2013.06.014","article-title":"Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients","volume":"19","author":"Dell\u2019Aquila","year":"2013","journal-title":"Park Relat Disord"},{"key":"2023080120203114100_awad105-B18","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1212\/WNL.38.7.1031","article-title":"Prevalence and natural history of progressive supranuclear palsy","volume":"38","author":"Golbe","year":"1988","journal-title":"Neurology"},{"key":"2023080120203114100_awad105-B19","doi-asserted-by":"crossref","first-page":"2301","DOI":"10.1002\/mds.28263","article-title":"Clinical conditions \u201csuggestive of progressive supranuclear palsy\u201d\u2014Diagnostic performance","volume":"35","author":"Grimm","year":"2020","journal-title":"Mov Disord"},{"key":"2023080120203114100_awad105-B20","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1016\/S1474-4422(14)70088-2","article-title":"Davunetide in patients with progressive supranuclear palsy: A randomised, double-blind, placebo-controlled phase 2\/3 trial","volume":"13","author":"Boxer","year":"2014","journal-title":"Lancet Neurol"},{"key":"2023080120203114100_awad105-B21","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1002\/mds.25824","article-title":"A phase 2 trial of the GSK-3 inhibitor tideglusib in progressive supranuclear palsy","volume":"29","author":"Tolosa","year":"2014","journal-title":"Mov Disord"},{"key":"2023080120203114100_awad105-B22","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/S1474-4422(19)30139-5","article-title":"Safety of the tau-directed monoclonal antibody BIIB092 in progressive supranuclear palsy: a randomised, placebo-controlled, multiple ascending dose phase 1b trial","volume":"18","author":"Boxer","year":"2019","journal-title":"Lancet Neurol"},{"key":"2023080120203114100_awad105-B23","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1038\/s41591-021-01455-x","article-title":"Safety and efficacy of anti-tau monoclonal antibody gosuranemab in progressive supranuclear palsy: a phase 2, randomized, placebo-controlled trial","volume":"27","author":"Dam","year":"2021","journal-title":"Nat Med"},{"key":"2023080120203114100_awad105-B24","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/S1474-4422(20)30489-0","article-title":"Safety and efficacy of tilavonemab in progressive supranuclear palsy: a phase 2, randomised, placebo-controlled trial","volume":"20","author":"H\u00f6glinger","year":"2021","journal-title":"Lancet Neurol"},{"key":"2023080120203114100_awad105-B25","doi-asserted-by":"crossref","first-page":"2129","DOI":"10.1002\/mds.22340","article-title":"Movement Disorder Society-sponsored revision of the Unified Parkinson\u2019s Disease Rating Scale (MDS-UPDRS): scale presentation and clinimetric testing results","volume":"23","author":"Goetz","year":"2008","journal-title":"Mov Disord"},{"key":"2023080120203114100_awad105-B26","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.1002\/mds.20255","article-title":"Development and validation of the Unified Multiple System Atrophy Rating Scale (UMSARS)","volume":"19","author":"Wenning","year":"2004","journal-title":"Mov Disord"},{"key":"2023080120203114100_awad105-B27","doi-asserted-by":"crossref","first-page":"1552","DOI":"10.1093\/brain\/awm032","article-title":"A clinical rating scale for progressive supranuclear palsy","volume":"130","author":"Golbe","year":"2007","journal-title":"Brain"},{"key":"2023080120203114100_awad105-B28","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1136\/jnnp-2016-314956","article-title":"Predictors of survival in progressive supranuclear palsy and multiple system atrophy: A systematic review and meta-analysis","volume":"88","author":"Glasmacher","year":"2017","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2023080120203114100_awad105-B29","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1007\/s00415-012-6624-1","article-title":"Expanded and independent validation of the Movement Disorder Society-Unified Parkinson\u2019s Disease Rating Scale (MDS-UPDRS)","volume":"260","author":"Martinez-Martin","year":"2013","journal-title":"J Neurol"},{"key":"2023080120203114100_awad105-B30","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1093\/brain\/awy319","article-title":"Longitudinal multimodal imaging and clinical endpoints for frontotemporal dementia clinical trials","volume":"142","author":"Staffaroni","year":"2019","journal-title":"Brain"},{"key":"2023080120203114100_awad105-B31","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1136\/jnnp-2021-326868","article-title":"Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort","volume":"93","author":"Peakman","year":"2022","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2023080120203114100_awad105-B32","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1002\/ana.25709","article-title":"Robust markers and sample sizes for multicenter trials of huntington disease","volume":"87","author":"Wijeratne","year":"2020","journal-title":"Ann Neurol"},{"key":"2023080120203114100_awad105-B33","doi-asserted-by":"crossref","first-page":"2547","DOI":"10.1038\/s41591-022-02075-9","article-title":"CSF Tau microtubule-binding region identifies pathological changes in primary tauopathies","volume":"28","author":"Horie","year":"2022","journal-title":"Nat Med"},{"key":"2023080120203114100_awad105-B34","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.parkreldis.2019.07.010","article-title":"Progression of two progressive supranuclear palsy phenotypes with comparable initial disability","volume":"66","author":"Shoeibi","year":"2019","journal-title":"Park Relat Disord"},{"key":"2023080120203114100_awad105-B35","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1007\/s00401-020-02158-2","article-title":"Distribution patterns of tau pathology in progressive supranuclear palsy","volume":"140","author":"Kovacs","year":"2020","journal-title":"Acta Neuropathol"},{"key":"2023080120203114100_awad105-B36","doi-asserted-by":"crossref","first-page":"923","DOI":"10.1136\/jnnp-2013-307035","article-title":"Validation of the new consensus criteria for the diagnosis of corticobasal degeneration","volume":"85","author":"Alexander","year":"2014","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2023080120203114100_awad105-B37","first-page":"1","article-title":"Mice: Multivariate imputation by chained equations in R","volume":"45","author":"van Buuren","year":"2011","journal-title":"J Stat Softw"},{"key":"2023080120203114100_awad105-B38","doi-asserted-by":"crossref","first-page":"1","DOI":"10.18637\/jss.v067.i01","article-title":"Fitting linear mixed-effects models using lme4","volume":"67","author":"Bates","year":"2015","journal-title":"J Stat Softw"},{"key":"2023080120203114100_awad105-B39","doi-asserted-by":"crossref","first-page":"742","DOI":"10.1002\/mds.26580","article-title":"Power calculations and placebo effect for future clinical trials in progressive supranuclear palsy","volume":"31","author":"Stamelou","year":"2016","journal-title":"Mov Disord"},{"key":"2023080120203114100_awad105-B40","author":"Therneau"},{"key":"2023080120203114100_awad105-B41","author":"Kassambara"},{"key":"2023080120203114100_awad105-B42","doi-asserted-by":"crossref","first-page":"603161","DOI":"10.3389\/fneur.2020.603161","article-title":"Association of MRI measures with disease severity and progression in progressive supranuclear palsy","volume":"11","author":"Picillo","year":"2020","journal-title":"Front Neurol"},{"key":"2023080120203114100_awad105-B43","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1016\/S1474-4422(12)70327-7","article-title":"The natural history of multiple system atrophy: A prospective European cohort study","volume":"12","author":"Wenning","year":"2013","journal-title":"Lancet Neurol"},{"key":"2023080120203114100_awad105-B44","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1016\/S1474-4422(15)00058-7","article-title":"Natural history of multiple system atrophy in the USA: A prospective cohort study","volume":"14","author":"Low","year":"2015","journal-title":"Lancet Neurol"},{"key":"2023080120203114100_awad105-B45","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s10286-021-00782-w","article-title":"Limitations of the unified multiple system atrophy rating scale as outcome measure for clinical trials and a roadmap for improvement","volume":"31","author":"Palma","year":"2021","journal-title":"Clin Auton Res"},{"key":"2023080120203114100_awad105-B46","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.parkreldis.2022.01.004","article-title":"Prediagnostic progressive supranuclear palsy\u2014Insights from the UK biobank","volume":"95","author":"Street","year":"2022","journal-title":"Park Relat Disord"},{"key":"2023080120203114100_awad105-B47","doi-asserted-by":"publisher","DOI":"10.1002\/alz.12802","article-title":"Pre-diagnostic cognitive and functional impairment in multiple sporadic neurodegenerative diseases","author":"Swaddiwudhipong","year":"12  2022","journal-title":"Alzheimer\u2019s Dement."},{"key":"2023080120203114100_awad105-B48","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.parkreldis.2020.03.003","article-title":"Prediagnostic motor and non-motor symptoms in progressive supranuclear palsy: the step-back PSP study","volume":"74","author":"Painous","year":"2020","journal-title":"Park Relat Disord"},{"key":"2023080120203114100_awad105-B49","doi-asserted-by":"crossref","first-page":"637176","DOI":"10.3389\/fneur.2021.637176","article-title":"Clinical features observed in general practice associated with the subsequent diagnosis of progressive supranuclear palsy","volume":"12","author":"Kwasny","year":"2021","journal-title":"Front Neurol"}],"container-title":["Brain"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/brain\/advance-article-pdf\/doi\/10.1093\/brain\/awad105\/49676771\/awad105.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/article-pdf\/146\/8\/3232\/51018545\/awad105.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/article-pdf\/146\/8\/3232\/51018545\/awad105.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,1]],"date-time":"2023-08-01T16:37:21Z","timestamp":1690907841000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/brain\/article\/146\/8\/3232\/7091433"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,28]]},"references-count":49,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2023,3,28]]},"published-print":{"date-parts":[[2023,8,1]]}},"URL":"https:\/\/doi.org\/10.1093\/brain\/awad105","relation":{},"ISSN":["0006-8950","1460-2156"],"issn-type":[{"value":"0006-8950","type":"print"},{"value":"1460-2156","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,8]]},"published":{"date-parts":[[2023,3,28]]}}}