{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T17:48:27Z","timestamp":1793382507617,"version":"4.5.0"},"reference-count":34,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2014,11,5]],"date-time":"2014-11-05T00:00:00Z","timestamp":1415145600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2014,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The phenotypic variability of progressive supranuclear palsy (PSP) may account for its frequent misdiagnosis, in particular in early stages of the disease. However, large multicenter studies to define the frequency and natural history of PSP phenotypes are missing. In a cohort of 100 autopsy\u2010confirmed patients we studied the phenotypic spectrum of PSP by retrospective chart review. Patients were derived from five brain banks with expertise in neurodegenerative disorders with referrals from multiple academic hospitals. The clinical characteristics of the 100 cases showed remarkable heterogeneity. Most strikingly, only 24% of cases presented as Richardson's Syndrome (RS), and more than half of the cases either showed overlapping features of several predescribed phenotypes, or features not fitting proposed classification criteria for PSP phenotypes. Classification of patients according to predominant clinical features in the first 2 years of the disease course allowed a more comprehensive description of the phenotypic spectrum. These predominance types differed significantly with regard to survival time and frequency of cognitive deficits. In summary, the phenotypic spectrum of PSP may be broader and more variable than previously described in single\u2010center studies. Thus, too strict clinical criteria defining distinct phenotypes may not reflect this variability. A more pragmatic clinical approach using predominance types could potentially be more helpful in the early recognition of and for making prognostic predictions for these patients. Given the limitations arising from the retrospective nature of this analysis, a systematic validation in a prospective cohort study is imperative. \u00a9 2014 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.26054","type":"journal-article","created":{"date-parts":[[2014,11,5]],"date-time":"2014-11-05T07:29:01Z","timestamp":1415172541000},"page":"1758-1766","source":"Crossref","is-referenced-by-count":372,"title":["The phenotypic spectrum of progressive supranuclear palsy: A retrospective multicenter study of 100 definite cases"],"prefix":"10.1002","volume":"29","author":[{"given":"Gesine","family":"Respondek","sequence":"first","affiliation":[{"name":"Department of Neurology Technische Universit\u00e4t M\u00fcnchen Munich Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) Munich Germany"},{"name":"Department of Neurology Philipps Universit\u00e4t Marburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Stamelou","sequence":"additional","affiliation":[{"name":"Department of Neurology Philipps Universit\u00e4t Marburg Germany"},{"name":"Second Department of Neurology Attiko Hospital, Kapodistrian University of Athens Athens Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolin","family":"Kurz","sequence":"additional","affiliation":[{"name":"Department of Neurology Technische Universit\u00e4t M\u00fcnchen Munich Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie W.","family":"Ferguson","sequence":"additional","affiliation":[{"name":"Division of Neurology Royal University Hospital, University of Saskatchewan  Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Rajput","sequence":"additional","affiliation":[{"name":"Division of Neurology Royal University Hospital, University of Saskatchewan  Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wan Zheng","family":"Chiu","sequence":"additional","affiliation":[{"name":"Department of Neurology Erasmus Medical Centre Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"van Swieten","sequence":"additional","affiliation":[{"name":"Department of Neurology Erasmus Medical Centre Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Troakes","sequence":"additional","affiliation":[{"name":"MRC London Neurodegenerative Diseases Brain Bank King's College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Safa","family":"al Sarraj","sequence":"additional","affiliation":[{"name":"MRC London Neurodegenerative Diseases Brain Bank King's College London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ellen","family":"Gelpi","sequence":"additional","affiliation":[{"name":"Neurological Tissue Bank and Neurology Department Hospital Cl\u00ednic de Barcelona, Universitat de Barcelona, IDIBAPS Barcelona Catalonia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carles","family":"Gaig","sequence":"additional","affiliation":[{"name":"Neurological Tissue Bank and Neurology Department Hospital Cl\u00ednic de Barcelona, Universitat de Barcelona, IDIBAPS Barcelona Catalonia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Tolosa","sequence":"additional","affiliation":[{"name":"Neurology Service Hospital Cl\u00ednic de Barcelona, Universitat de Barcelona, IDIBAPS, Centro de Investigaci\u00f3n Biom\u00e9dica en Red sobre Enfermedades Neurodegenerativas (CIBERNED) Barcelona Catalonia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang H.","family":"Oertel","sequence":"additional","affiliation":[{"name":"Department of Neurology Philipps Universit\u00e4t Marburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Armin","family":"Giese","sequence":"additional","affiliation":[{"name":"Center for Neuropathology and Prion Research Ludwig Maximilians University Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sigrun","family":"Roeber","sequence":"additional","affiliation":[{"name":"Center for Neuropathology and Prion Research Ludwig Maximilians University Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Arzberger","sequence":"additional","affiliation":[{"name":"Center for Neuropathology and Prion Research Ludwig Maximilians University Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Wagenpfeil","sequence":"additional","affiliation":[{"name":"Department of Medical Statistics and Epidemiology Technische Universit\u00e4t M\u00fcnchen Munich Germany"},{"name":"Insitute for Medical Biometry Epidemiology and Medical Informatics, Universit\u00e4tsklinikum des Saarlandes  Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00fcnter U.","family":"H\u00f6glinger","sequence":"additional","affiliation":[{"name":"Department of Neurology Technische Universit\u00e4t M\u00fcnchen Munich Germany"},{"name":"German Center for Neurodegenerative Diseases (DZNE) Munich Germany"},{"name":"Department of Neurology Philipps Universit\u00e4t Marburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the Movement Disorder Society\u2014endorsed PSP Study 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