W R Wayne Martin1, Michael Miles2, Qiaonan Zhong3, Johanna Hartlein2, Brad A Racette2,4, Scott A Norris2,5, Mwiza Ushe2, Baijayanta Maiti2, Susan Criswell2, Albert A Davis2, Paul T Kotzbauer2, Nigel J Cairns6, Richard J Perrin2,7, Joel S Perlmutter2,5,8. 1. Department of Medicine (Neurology), University of Alberta, Edmonton, Alberta, Canada. 2. Department of Neurology, Washington University in St. Louis, St. Louis, Missouri, USA. 3. Department of Medicine, Mayo Clinic, Rochester, Missouri, USA. 4. Faculty of Health Sciences, School of Public Health, University of the Witwatersrand, Parktown, South Africa. 5. Department of Radiology, Washington University in St. Louis, St. Louis, Missouri, USA. 6. College of Medicine and Health, University of Exeter, Exeter, United Kingdom. 7. Department of Pathology and Immunology, Washington University in St. Louis, St. Louis, Missouri, USA. 8. Departments of Neuroscience, Physical Therapy and Occupational Therapy, Washington University in St. Louis, St. Louis, Missouri, USA.
Abstract
BACKGROUND: The clinical diagnosis of Parkinson's disease (PD) requires the presence of parkinsonism and supportive criteria that include a clear and dramatic beneficial response to dopaminergic therapy. Our aim was to test the diagnostic criterion of dopaminergic response by evaluating its association with pathologically confirmed diagnoses in a large population of parkinsonian patients. METHODS: We reviewed clinical data maintained in an electronic medical record from all patients with autopsy data who had been seen in the Movement Disorders Center at Washington University, St. Louis, between 1996 and 2018. All patients with parkinsonism who underwent postmortem neuropathologic examination were included in this analysis. RESULTS: There were 257 unique parkinsonian patients with autopsy-based diagnoses who had received dopaminergic therapy. Marked or moderate response to dopaminergic therapy occurred in 91.2% (166/182) of those with autopsy-confirmed PD, 52.0% (13/25) of those with autopsy-confirmed multiple systems atrophy, 44.4% (8/18) of those with autopsy-confirmed progressive supranuclear palsy, and 1 (1/8) with autopsy-confirmed corticobasal degeneration. Other diagnoses were responsible for the remaining 24 individuals, 9 of whom had a moderate response to dopaminergic therapy. CONCLUSION: A substantial response to dopaminergic therapy is frequent but not universal in PD. An absent response does not exclude PD. In other neurodegenerative disorders associated with parkinsonism, a prominent response may also be evident, but this occurs less frequently than in PD.
BACKGROUND: The clinical diagnosis of Parkinson's disease (PD) requires the presence of parkinsonism and supportive criteria that include a clear and dramatic beneficial response to dopaminergic therapy. Our aim was to test the diagnostic criterion of dopaminergic response by evaluating its association with pathologically confirmed diagnoses in a large population of parkinsonian patients. METHODS: We reviewed clinical data maintained in an electronic medical record from all patients with autopsy data who had been seen in the Movement Disorders Center at Washington University, St. Louis, between 1996 and 2018. All patients with parkinsonism who underwent postmortem neuropathologic examination were included in this analysis. RESULTS: There were 257 unique parkinsonian patients with autopsy-based diagnoses who had received dopaminergic therapy. Marked or moderate response to dopaminergic therapy occurred in 91.2% (166/182) of those with autopsy-confirmed PD, 52.0% (13/25) of those with autopsy-confirmed multiple systems atrophy, 44.4% (8/18) of those with autopsy-confirmed progressive supranuclear palsy, and 1 (1/8) with autopsy-confirmed corticobasal degeneration. Other diagnoses were responsible for the remaining 24 individuals, 9 of whom had a moderate response to dopaminergic therapy. CONCLUSION: A substantial response to dopaminergic therapy is frequent but not universal in PD. An absent response does not exclude PD. In other neurodegenerative disorders associated with parkinsonism, a prominent response may also be evident, but this occurs less frequently than in PD.
Authors: Ronald B Postuma; Daniela Berg; Matthew Stern; Werner Poewe; C Warren Olanow; Wolfgang Oertel; José Obeso; Kenneth Marek; Irene Litvan; Anthony E Lang; Glenda Halliday; Christopher G Goetz; Thomas Gasser; Bruno Dubois; Piu Chan; Bastiaan R Bloem; Charles H Adler; Günther Deuschl Journal: Mov Disord Date: 2015-10 Impact factor: 10.338
Authors: S Gilman; S J May; C W Shults; C M Tanner; W Kukull; V M-Y Lee; E Masliah; P Low; P Sandroni; J Q Trojanowski; L Ozelius; T Foroud Journal: J Neural Transm (Vienna) Date: 2005-12 Impact factor: 3.575
Authors: I G McKeith; D Galasko; K Kosaka; E K Perry; D W Dickson; L A Hansen; D P Salmon; J Lowe; S S Mirra; E J Byrne; G Lennox; N P Quinn; J A Edwardson; P G Ince; C Bergeron; A Burns; B L Miller; S Lovestone; D Collerton; E N Jansen; C Ballard; R A de Vos; G K Wilcock; K A Jellinger; R H Perry Journal: Neurology Date: 1996-11 Impact factor: 9.910
Authors: David R Williams; Rohan de Silva; Dominic C Paviour; Alan Pittman; Hilary C Watt; Linda Kilford; Janice L Holton; Tamas Revesz; Andrew J Lees Journal: Brain Date: 2005-03-23 Impact factor: 13.501
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