@article{cfc06da8862e4a108ba491869670d78c,
title = "Is Levodopa Response a Valid Indicator of Parkinson's Disease?",
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.",
keywords = "Parkinson's disease, diagnostic specificity, levodopa, parkinsonism",
author = "Martin, {W. R.Wayne} and Michael Miles and Qiaonan Zhong and Johanna Hartlein and Racette, {Brad A.} and Norris, {Scott A.} and Mwiza Ushe and Baijayanta Maiti and Susan Criswell and Davis, {Albert A.} and Kotzbauer, {Paul T.} and Cairns, {Nigel J.} and Perrin, {Richard J.} and Perlmutter, {Joel S.}",
note = "Funding Information: Baijayanta Maiti has received funding from the National Center for Advancing Translational Sciences of the National Institute of Health KL2 TR002346, American Academy of Neurology and American Brain Foundation Clinical Research Training Fellowship in Parkinson's Disease, Parkinson Study Group, Parkinson's Disease Foundation Mentored Clinical Research Award, the Jo Oertli Fund, and the Greater St. Louis Chapter of American Parkinson Disease Association. Funding Information: Scott A. Norris receives funds from NIH R01 NS107281 and RO1 NS103957 (Perlmutter), TR00145609 (Jinnah), Scott G. Lentin fund, and Dystonia Medical Research Foundation. Funding Information: Richard J. Perrin has received research funding from the National Institutes of Health AG024904, AG003991, AG005681, R01 NS075321, R01 NS092865, R01 NS097799, RF1 AG053550, R01 AG054513, R01 AG054567, R01 AG052550, U19 AG032438, and UF1 AG032438; American Parkinson Disease Association (Advanced Research Center for Parkinson Disease); BrightFocus Foundation—Alzheimer Grant Award A2014296S; and Janssen Biotech, Inc. (in support of work as Dominantly Inherited Alzheimer Network—Trials Unit Neuropathology Core Leader). Funding Information: Albert A. Davis has received research funding from NIH NS101118, American Academy of Neurology/American Brain Foundation, BrightFocus Foundation, Mary E. Groff Charitable Trust, and Dobbins Family Fund. Funding Information: Brad A. Racette receives research support from NIEHS grants R01ES026891, R01ES026891‐S1, R01ES025991, R01ES025991‐02S1, grant 10289‐01 Michael J. Fox Foundation, American Parkinson Disease Association, Hope Center for Neurologic Disorders, Cure Alzheimer's Fund, R01ES030937‐S1, R01ES029524, and ROH01166. Dr. Racette has received honoraria (personal compensation) for a lecture from the University of Michigan, for peer review from the Parkinson Study Group, for service on the National Advisory Environmental Health Sciences Council for NIEHS, and for medical expert testimony in the last year, unrelated to the topic of this manuscript. Funding Information: agencies: This study was supported by grants from the National Institutes of Health (NS07532, NS097799, U10NS077384), the American Parkinson Disease Association (APDA), the Greater St. Louis Chapter of the APDA, the Jo Oertli Fund, the Barnes Jewish Hospital Foundation (Elliot Stein Family Fund and the Parkinson Disease Research Fund), and the Paula and Rodger Riney Fund.This work was supported by grants from the National Institutes of Health (NS07532, NS097799, U10NS077384), the American Parkinson Disease Association (APDA), the Greater St. Louis Chapter of the APDA, the Jo Oertli Fund, the Barnes Jewish Hospital Foundation (Elliot Stein Family Fund and the Parkinson Disease Research Fund), and the Paula and Rodger Riney Fund. We thank our patients and families without whom this research would not be possible. Funding Information: Joel S. Perlmutter has received research funding from NIH NS075321, NS103957, NS107281, NS092865, U10NS077384, NS097437, U54NS116025, U19 NS110456, AG050263, AG‐64937, NS097799, NS075527, ES029524, NS109487, and R61 AT010753, (National Center for Advancing Translational Sciences [NCATS], National Institute of Neurological Disorders and Stroke [NINDS], NIA); Department of Defense (DOD W81XWH‐217‐1‐0393); Michael J. Fox Foundation; Barnes‐Jewish Hospital Foundation (Elliot Stein Family Fund and Parkinson Disease Research Fund); American Parkinson Disease Association (APDA) Advanced Research Center at Washington University; Greater St. Louis Chapter of the APDA; Paula and Rodger Riney Fund; Jo Oertli Fund; Huntington's Disease Society of America; Murphy Fund; and CHDI. He received honoraria from CHDI, Huntington Study Group, Parkinson Study Group, Beth Israel Hospital (Harvard group), U Pennsylvania, and Stanford U. He also is codirector of the Dystonia Coalition, which has received the majority of its support through the NIH (grants NS116025 and NS065701 from the National Institutes of Neurological Disorders and Stroke TR 001456 from the Office of Rare Diseases Research at the National Center for Advancing Translational Sciences). Dr. Perlmutter serves as director of the Medical and Scientific Advisory Committee of the Dystonia Medical Research Foundation, chair of the Scientific Advisory Committee of the Parkinson Study Group, chair of the Standards Committee of the Huntington Study Group, member of the Scientific Advisory Board of the APDA, chair of the Scientific and Publication Committee for ENROLL‐HD, and member of the Education Committee of the Huntington Study Group. He has provided medical legal consultation to Wood, Cooper and Peterson, LLC, and to Simmons and Simmons LLP. Funding Information: Johanna Hartlein has received funding from NIH NS075321, NS097437, and NS097799. Funding Information: Michael Miles has received funding from a NIH T35 NHLBI Training Grant. Funding Information: Susan Criswell receives research support from NIH grants R01 ES026891, R01 ES029524, and R01 OH011661. Funding Information: This work was supported by grants from the National Institutes of Health (NS07532, NS097799, U10NS077384), the American Parkinson Disease Association (APDA), the Greater St. Louis Chapter of the APDA, the Jo Oertli Fund, the Barnes Jewish Hospital Foundation (Elliot Stein Family Fund and the Parkinson Disease Research Fund), and the Paula and Rodger Riney Fund. We thank our patients and families without whom this research would not be possible. Funding Information: Paul T. Kotzbauer receives research support from the following: NIH grants U01 NS110436, R01 NS097799, R01 NS097437, R21 NS109831, R01 NS075321, R01 AG050263, Michael J. Fox Foundation, Alzheimer's Association, ALS Finding a Cure, and ALS Association. He also receives research support from INADcure Foundation, Jasper Valentijn Foundation, Biogen, and AbbVie. Publisher Copyright: {\textcopyright} 2020 International Parkinson and Movement Disorder Society",
year = "2021",
month = apr,
doi = "10.1002/mds.28406",
language = "English",
volume = "36",
pages = "948--954",
journal = "Movement Disorders",
issn = "0885-3185",
number = "4",
}