Max-Joseph Grimm1,2, Gesine Respondek2,3, Maria Stamelou4,5,6, Thomas Arzberger2,7,8, Leslie Ferguson9, Ellen Gelpi10,11, Armin Giese7, Murray Grossman12, David J Irwin12, Alexander Pantelyat13, Alex Rajput9, Sigrun Roeber7, John C van Swieten14, Claire Troakes15, Wassilios G Meissner16,17,18, Christer Nilsson19, Ines Piot1,2, Yaroslau Compta20,21,22, James B Rowe23, Günter U Höglinger1,2,3. 1. Department of Neurology, Technische Universität München, Munich, Germany. 2. German Center for Neurodegenerative Diseases (DZNE), Munich, Germany. 3. Department of Neurology, Hannover Medical School, Hannover, Germany. 4. Parkinson's Disease and Movement Disorders Department, HYGEIA Hospital, National and Kapodistrian University of Athens, Athens, Greece. 5. First Department of Neurology, Aiginiteion Hospital, National and Kapodistrian University of Athens, Athens, Greece. 6. Department of Neurology, Philipps Universität, Marburg, Germany. 7. Center for Neuropathology and Prion Research, Ludwig-Maximilians-Universität, Munich, Germany. 8. Department of Psychiatry and Psychotherapy, University Hospital, LMU Munich, Munich, Germany. 9. Division of Neurology, Royal University Hospital, University of Saskatchewan, Saskatoon, Saskatchewan, Canada. 10. Neurological Tissue Bank and Neurology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, IDIBAPS, CERCA, Barcelona, Spain. 11. Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria. 12. Frontotemporal Degeneration Center, Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA. 13. Department of Neurology, Johns Hopkins University, Baltimore, Maryland, USA. 14. Department of Neurology, Erasmus Medical Centre, Rotterdam, The Netherlands. 15. London Neurodegenerative Diseases Brain Bank, Institute of Psychiatry, Psychology and Neuroscience, Kings College London, London, UK. 16. University de Bordeaux, Institut des Maladies Neurodégénératives, CNRS UMR 5293, Bordeaux, France. 17. Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, Bordeaux, France. 18. University of Otago, Christchurch, and New Zealand Brain Research Institute, Department Medicine, Christchurch, New Zealand. 19. Department of Clinical Sciences, Division of Neurology, Lund University, Lund, Sweden. 20. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Hospital Clínic de Barcelona, Barcelona, Spain. 21. Movement Disorders Unit, Neurology Service, Hospital Clínic de Barcelona. Institute of Neuroscience, University of Barcelona, Barcelona, Spain. 22. Institute of Biomedical Research August Pi i Sunyer (IDIBAPS), Barcelona, Spain. 23. Department of Clinical Neurosciences and Cambridge Centre for Parkinson-Plus, Cambridge University, Cambridge, UK.
Abstract
BACKGROUND: The Movement Disorder Society diagnostic criteria for progressive supranuclear palsy introduced the diagnostic certainty level "suggestive of progressive supranuclear palsy" for clinical conditions with subtle signs, suggestive of the disease. This category aims at the early identification of patients, in whom the diagnosis may be confirmed as the disease evolves. OBJECTIVE: To assess the diagnostic performance of the defined clinical conditions suggestive of progressive supranuclear palsy in an autopsy-confirmed cohort. METHODS: Diagnostic performance of the criteria was analyzed based on retrospective clinical data of 204 autopsy-confirmed patients with progressive supranuclear palsy and 216 patients with other neurological diseases. RESULTS: The conditions suggestive of progressive supranuclear palsy strongly increased the sensitivity compared to the National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy criteria. Within the first year after symptom onset, 40% of patients with definite progressive supranuclear palsy fulfilled criteria for suggestive of progressive supranuclear palsy. Two-thirds of patients suggestive of progressive supranuclear palsy evolved into probable progressive supranuclear palsy after an average of 3.6 years. Application of the criteria for suggestive of progressive supranuclear palsy reduced the average time to diagnosis from 3.8 to 2.2 years. CONCLUSIONS: Clinical conditions suggestive of progressive supranuclear palsy allow earlier identification of patients likely to evolve into clinically possible or probable progressive supranuclear and to have underlying progressive supranuclear palsy pathology. Further work needs to establish the specificity and positive predictive value of this category in real-life clinical settings, and to develop specific biomarkers that enhance their diagnostic accuracy in early disease stages.
BACKGROUND: The Movement Disorder Society diagnostic criteria for progressive supranuclear palsy introduced the diagnostic certainty level "suggestive of progressive supranuclear palsy" for clinical conditions with subtle signs, suggestive of the disease. This category aims at the early identification of patients, in whom the diagnosis may be confirmed as the disease evolves. OBJECTIVE: To assess the diagnostic performance of the defined clinical conditions suggestive of progressive supranuclear palsy in an autopsy-confirmed cohort. METHODS: Diagnostic performance of the criteria was analyzed based on retrospective clinical data of 204 autopsy-confirmed patients with progressive supranuclear palsy and 216 patients with other neurological diseases. RESULTS: The conditions suggestive of progressive supranuclear palsy strongly increased the sensitivity compared to the National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy criteria. Within the first year after symptom onset, 40% of patients with definite progressive supranuclear palsy fulfilled criteria for suggestive of progressive supranuclear palsy. Two-thirds of patients suggestive of progressive supranuclear palsy evolved into probable progressive supranuclear palsy after an average of 3.6 years. Application of the criteria for suggestive of progressive supranuclear palsy reduced the average time to diagnosis from 3.8 to 2.2 years. CONCLUSIONS: Clinical conditions suggestive of progressive supranuclear palsy allow earlier identification of patients likely to evolve into clinically possible or probable progressive supranuclear and to have underlying progressive supranuclear palsy pathology. Further work needs to establish the specificity and positive predictive value of this category in real-life clinical settings, and to develop specific biomarkers that enhance their diagnostic accuracy in early disease stages.
Authors: Thomas W Rösler; Amir Tayaranian Marvian; Matthias Brendel; Niko-Petteri Nykänen; Matthias Höllerhage; Sigrid C Schwarz; Franziska Hopfner; Thomas Koeglsperger; Gesine Respondek; Kerstin Schweyer; Johannes Levin; Victor L Villemagne; Henryk Barthel; Osama Sabri; Ulrich Müller; Wassilios G Meissner; Gabor G Kovacs; Günter U Höglinger Journal: Prog Neurobiol Date: 2019-06-22 Impact factor: 11.685
Authors: Farwa Ali; Peter R Martin; Hugo Botha; J Eric Ahlskog; James H Bower; Joseph Y Masumoto; Demetrius Maraganore; Anhar Hassan; Scott Eggers; Bradley F Boeve; David S Knopman; Daniel Drubach; Ronald C Petersen; Erika Driver Dunkley; Jay van Gerpen; Ryan Uitti; Jennifer L Whitwell; Dennis W Dickson; Keith A Josephs Journal: Mov Disord Date: 2019-02-06 Impact factor: 10.338
Authors: I Litvan; Y Agid; D Calne; G Campbell; B Dubois; R C Duvoisin; C G Goetz; L I Golbe; J Grafman; J H Growdon; M Hallett; J Jankovic; N P Quinn; E Tolosa; D S Zee Journal: Neurology Date: 1996-07 Impact factor: 9.910
Authors: J J Hauw; S E Daniel; D Dickson; D S Horoupian; K Jellinger; P L Lantos; A McKee; M Tabaton; I Litvan Journal: Neurology Date: 1994-11 Impact factor: 9.910
Authors: Gesine Respondek; Carolin Kurz; Thomas Arzberger; Yaroslau Compta; Elisabet Englund; Leslie W Ferguson; Ellen Gelpi; Armin Giese; David J Irwin; Wassilios G Meissner; Christer Nilsson; Alexander Pantelyat; Alex Rajput; John C van Swieten; Claire Troakes; Keith A Josephs; Anthony E Lang; Brit Mollenhauer; Ulrich Müller; Jennifer L Whitwell; Angelo Antonini; Kailash P Bhatia; Yvette Bordelon; Jean-Christophe Corvol; Carlo Colosimo; Richard Dodel; Murray Grossman; Jan Kassubek; Florian Krismer; Johannes Levin; Stefan Lorenzl; Huw Morris; Peter Nestor; Wolfgang H Oertel; Gil D Rabinovici; James B Rowe; Thilo van Eimeren; Gregor K Wenning; Adam Boxer; Lawrence I Golbe; Irene Litvan; Maria Stamelou; Günter U Höglinger Journal: Mov Disord Date: 2017-05-13 Impact factor: 10.338
Authors: Günter U Höglinger; Gesine Respondek; Maria Stamelou; Carolin Kurz; Keith A Josephs; Anthony E Lang; Brit Mollenhauer; Ulrich Müller; Christer Nilsson; Jennifer L Whitwell; Thomas Arzberger; Elisabet Englund; Ellen Gelpi; Armin Giese; David J Irwin; Wassilios G Meissner; Alexander Pantelyat; Alex Rajput; John C van Swieten; Claire Troakes; Angelo Antonini; Kailash P Bhatia; Yvette Bordelon; Yaroslau Compta; Jean-Christophe Corvol; Carlo Colosimo; Dennis W Dickson; Richard Dodel; Leslie Ferguson; Murray Grossman; Jan Kassubek; Florian Krismer; Johannes Levin; Stefan Lorenzl; Huw R Morris; Peter Nestor; Wolfgang H Oertel; Werner Poewe; Gil Rabinovici; James B Rowe; Gerard D Schellenberg; Klaus Seppi; Thilo van Eimeren; Gregor K Wenning; Adam L Boxer; Lawrence I Golbe; Irene Litvan Journal: Mov Disord Date: 2017-05-03 Impact factor: 10.338
Authors: Fatma Ozlem Hokelekli; Joseph R Duffy; Heather M Clark; Rene L Utianski; Hugo Botha; Farwa Ali; Julie A Stierwalt; Mary M Machulda; R Ross Reichard; Dennis W Dickson; Jennifer L Whitwell; Keith A Josephs Journal: Mov Disord Date: 2021-10-10 Impact factor: 10.338
Authors: Maria Stamelou; Gesine Respondek; Nikolaos Giagkou; Jennifer L Whitwell; Gabor G Kovacs; Günter U Höglinger Journal: Nat Rev Neurol Date: 2021-08-23 Impact factor: 42.937
Authors: Meg E Morris; Susan C Slade; Christopher Bruce; Jennifer L McGinley; Bastiaan R Bloem Journal: Front Neurol Date: 2021-02-02 Impact factor: 4.003
Authors: Alejandra Martínez-Maldonado; Miguel Ángel Ontiveros-Torres; Charles R Harrington; José Francisco Montiel-Sosa; Raúl García-Tapia Prandiz; Patricia Bocanegra-López; Andrew Michael Sorsby-Vargas; Marely Bravo-Muñoz; Benjamín Florán-Garduño; Ignacio Villanueva-Fierro; George Perry; Linda Garcés-Ramírez; Fidel de la Cruz; Sandra Martínez-Robles; Mar Pacheco-Herrero; José Luna-Muñoz Journal: J Alzheimers Dis Date: 2021 Impact factor: 4.472
Authors: Duncan Street; Maura Malpetti; Timothy Rittman; Boyd C P Ghosh; Alexander G Murley; Ian Coyle-Gilchrist; Luca Passamonti; James B Rowe Journal: Brain Commun Date: 2021-09-02
Authors: Javier Sánchez-Ruiz de Gordoa; Victoria Zelaya; Paula Tellechea-Aramburo; Blanca Acha; Miren Roldán; Carlos López-Molina; Valle Coca; Arkaitz Galbete; Maite Mendioroz; M Elena Erro Journal: Front Neurol Date: 2022-02-03 Impact factor: 4.003
Authors: Andrea Quattrone; Maurizio Morelli; Maria G Bianco; Jolanda Buonocore; Alessia Sarica; Maria Eugenia Caligiuri; Federica Aracri; Camilla Calomino; Marida De Maria; Maria Grazia Vaccaro; Vera Gramigna; Antonio Augimeri; Basilio Vescio; Aldo Quattrone Journal: Brain Sci Date: 2022-07-20