How Do I Diagnose Multiple System Atrophy—A Videolibrary on Clinical and Imaging Features

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder clinically manifesting with autonomic failure, parkinsonism, and cerebellar syndrome in various combinations. Although a definite diagnosis of MSA can be achieved only neuropathologically by identifying glial cytoplasmic inclusions containing α-synuclein, the Movement Disorder Society (MDS)-MSA diagnostic criteria allow for diagnosing MSA during lifetime.1 These criteria consider different levels of diagnostic accuracy including clinically established MSA with maximum specificity of 99% to 100% and clinically probable MSA with balanced sensitivity (64%–77%) and specificity (74%–100%).2-4 Research category of possible prodromal MSA aims at identifying patients in the earliest disease stage when non-motor symptoms predominate, who could benefit most from the inclusion in clinical trials with disease modifying drugs. The aim of this “How Do I” video project is to support clinicians in using the MDS-MSA criteria by showing videos for each feature of the criteria. Before each feature, a slide shows in what category this criteria falls. Video 1 presents typical core features (autonomic failure, parkinsonism, and cerebellar syndrome) and supportive motor and non-motor features (previously termed MSA “red flags”) of clinically established and clinically probable MSA. Characteristic radiological findings of MSA required only for clinically established diagnosis are also given in the Video 1. Video 2 demonstrates progression from the diagnosis of possible prodromal MSA to the diagnosis of clinically established MSA. (1) Research project: A. Conception, B. Organization, C. Execution; (2) Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique; V.S.: 1A, 1B, 1C, 2A L.B.: 1B, 1C, 2B N.B.: 1B, 1C, 2B G.C.B.: 1B, 1C, 2B N.C.: 1B, 1C, 2B G.D.P.: 1B, 1C, 2B M.F.: 1B, 1C, 2B A.F.: 1B, 1C, 2B J.F.: 1B, 1C, 2B F.G.: 1B, 1C, 2B E.G.: 1B, 1C, 2B G.G.: 1B, 1C, 2B S.K.: 1B, 1C, 2B H.K.: 1B, 1C, 2B V.K.: 1B, 1C, 2B F.K.: 1B, 1C, 2B A.L.: 1B, 1C, 2B J.L.: 1B, 1C, 2B P.M.V.: 1B, 1C, 2B M.P.: 1B, 1C, 2B I.P.: 1B, 1C, 2B W.P.: 1B, 1C, 2B C.R.: 1B, 1C, 2B R.M.S.: 1B, 1C, 2B W.S.: 1B, 1C, 2B M.Str.: 1B, 1C, 2B T.V.E.: 1B, 1C, 2B M.Sta.: 1A, 1B, 1C, 3B G.H.: 1A, 1B, 1C, 3B G.W.: 1A, 1B, 1C, 3B I.S.: 1A, 1B, 1C, 2B We thank our patients for taking part in this study and accepting their videos to be published for educational purposes. Open access funding provided by Medizinische Universitat Innsbruck/KEMÖ. Ethical Compliance Statement: The study protocol was approved by the local ethics committees where needed. All patients gave written informed consent before their participation, and study staff provided written Declaration of investigator for each video and image proving that their patients consented to video/magnetic resonance imaging recording and publication. The examinations were carried out in accordance with the principles expressed in the Declaration of Helsinki. On behalf of all co-authors, the first and corresponding author confirm that all authors have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. Funding Sources and Conflict of Interest: No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work. Financial Disclosures for the previous 12 months: M.F. received honoraria to speak from AbbVie, ORKYN, and BIAL, consultancies from BIAL and LVL Medical; additionally she received grants from France Parkinson, French Health Ministry and MSA Coalition. A.F. reports royalties from Springer Verlag, speaker fees and honoraria from Bial, CNSystems, Theravance Biopharma, GE Health Care, Broadview Ventures, Austrian Autonomic Society, Elsevier and research grants from the FWF-Austrian Science Fund, Medical University of Innsbruck, US MSA Coalition, Dr Johannes and Hertha Tuba Foundation and Austrian Exchange Program, outside of the present work. J.F. has provided consultancy and received speaker fees from Bial, Biogen, AbbVie, Sunovion Pharmaceuticals, Infucure, Zambon, Roche, Stada, ONO Pharma, Britannia, Asta, Neuroderm, and SK Chemicals; and has received grants from AbbVie, Bial, Medtronic and Angelini. F.G. received speaker honoraria from AbbVie, Bial, Campus, Stada; and serves at the Advisory Board of AbbVie and Stada. E.G. provided consultancies from Bago Argentina, Biogen, UCB; and serves at the Advisory Board of Bago, UCB, and Europharma; she received honoraria as speaker from Megalabs, Bago, UCB, IPMDS, and IAPRDS for lectures, and from Roche lab and ENROLL-HD for clinical trials. E.G. received honoraria as Co-editor of P&RD Journal and grants from the National institute Yerba Mate. S.K. has received support from CurePSP, ERN-RND, Deutsche Forschungsgemeinschaft (DFG) under Germany's Excellence Strategy within the framework of the Munich Cluster for Systems Neurology (EXC 2145 SyNergy; ID 390857198). H.K. is Editor-in-Chief of Clinical Autonomic Research, published by Springer-Nature, serves as PI of studies sponsored by Biogen MA. (TRACK MSA, S19-01846) and Vaxxinity (UB-312, S22-01332). He has received consultant fees from Teva Pharmaceutical Company, Theravance Biopharma US, and Parexel. He receives royalties from Up To Date. He receives research support from NIH-NINDS, Food and Drug Administration, Familial Dysautonomia Foundation, and HSAN IV Foundation. F.K. received personal fees from Institut de Recherches Internationales Servier, Takeda Pharmaceuticals, Sanofi, Teva, Bial, and the Austrian Society of Neurology in the past 36 months; and he has ongoing grant support from the Austrian Science Fund (FWF), the National Institutes of Health and The Michael J. Fox Foundation, outside of the submitted work. V.K. is a co-founder and senior advisor to DaCapo Brainscience, a company focused on CNS diseases. He has served as a consultant for Sanofi, AC Immune, and Yoda Pharmaceuticals. A.L. has served as an advisor for AbbVie, Amylyx, Aprinoia, Biogen, BioAdvance, Biohaven, BioVie, BlueRock, BMS, Denali, Janssen, Lilly, Pharma 2B, Sun Pharma, and UCB; received honoraria from Sun Pharma, AbbVie, and Sunovion; received grants from Brain Canada, Canadian Institutes of Health Research, Edmond J Safra Philanthropic Foundation, The Michael J. Fox Foundation, the Ontario Brain Institute, Parkinson Foundation, Parkinson Canada, and W. Garfield Weston Foundation; is serving as an expert witness in litigation related to paraquat and Parkinson's disease, received publishing royalties from Elsevier, Saunders, Wiley-Blackwell, Johns Hopkins Press, and Cambridge University Press. J.L. reports speaker fees from Bayer Vital, Biogen, EISAI, Esteve, and Roche, consulting fees from Axon Neuroscience and Biogen, author fees from Thieme medical publishers and W. Kohlhammer medical publishers, compensation for serving as chief medical officer for MODAG. J.L. is beneficiary of the phantom share program of MODAG and is inventor in a patent “Pharmaceutical Composition and Methods of Use” (EP 22159408.8) filed by MODAG, all activities outside the submitted work. I.P. received speaker honoraria from Mak Pharma, Salveo, and Inopharm. R.M.S. served as consultant for BIAL-PORTELA and C.a, S.A.; in advisory boards for AbbVie and STADA; received honoraria as a speaker for AbbVie and BIAL-PORTELA & C.a, S.A. M.Str. is Joint Chief Editor of the Journal of Neurology, Editor in Chief of Frontiers of Neuro-otology and Section Editor of F1000. He has received speaker's honoraria from Abbott, Auris Medical, Biogen, Eisai, Grünenthal, GSK, Henning Pharma, Interacoustics, J&J, MSD, NeuroUpdate, Otometrics, Pierre-Fabre, TEVA, UCB, and Viatris. He receives support for clinical studies from Decibel, US, Cure within Reach, US. and Heel, Germany. He distributes “M-glasses” and “Positional vertigo App.” He acts as a consultant for Abbott, AurisMedical, Bulbitec, Heel, IntraBio, Sensorion, Vifor, and Vertify. He is investor and shareholder of IntraBio. T.v.E. has received support from ICON plc and GT Gain Therapeutics SA. M.Sta. received funding from the ‘Non-profit Organization for Research in Parkinson's disease and Related Disorders’, consultancies from UCB, Roche, ERC; and speaker and travel honoraria from MDS, EAN. G.H. was funded by the DFG (German Research Foundation) under Germany's Excellence Strategy within the framework of the Munich Cluster for Systems Neurology (EXC 2145 SyNergy—ID 390857198), DFG (HO2402/18-1 MSAomics); has ongoing research collaborations with Roche, UCB, AbbVie; served as a consultant for AbbVie, Alzprotect, Amylyx, Aprinoia, Asceneuron, Bayer, Bial, Biogen, Biohaven, Epidarex, Ferrer, Kyowa Kirin, Lundbeck, Novartis, Retrotope, Roche, Sanofi, Servier, Takeda, Teva, and UCB; received honoraria for scientific presentations from AbbVie, Bayer, Bial, Biogen, Bristol Myers Squibb, Esteve, Kyowa Kirin, Pfizer, Roche, Teva, UCB, and Zambon; holds a patent on the Treatment of Synucleinopathies (United States patent US 10,918,628 B2/European patent EP 17787904.6-1109/3,525,788); received publication royalties from Academic Press, Kohlhammer, and Thieme. A.A. has received compensation for consultancy and speaker-related activities from UCB, Britannia, AbbVie, Zambon, Bial, Neuroderm, Theravance Biopharma, and Roche. He receives research support from AbbVie, Bial, Horizon2020, Italian Ministry of University and Research (MUR), Italian Ministry of Health (MOH), and Fondazione Grigioni. T.G. received research funding from the Charitable Hertie Foundation, the German Research Foundation and the European Commission. He also received speakers honoraria from MedUpdate, Sanofi, UCB, and Desitin. He has received consulting fees from Bayer AG, BIAL, Blue Rock Therapeutics, Roche Pharma AG, and Lilly Deutschland; and received the Steering Committee Member Award from The Michael J. Fox Foundation for Parkinson's Research. He is also chairman of the scientific advisory board of the “Joint Programming for Neurodegenerative Diseases”, funded by the European Commission. T.G. reports consultancy fees from AbbVie, Neuroderm, and Tradis Gat; received speakers fee from Medison; received travel support for her and her team from Alphamedix, AbbVie, and Medison; and grants from International Parkinson Disease and Movement Disorders Society and Parkinson's Foundation, outside of the present work. N.M. has received research funding from the NIH, The Michael J. Fox Foundation, and Parkinson Foundation; receives royalties from Wolters Kluwer Health; and serves as consultant or steering committee member for ONO Pharmaceuticals. B.R. was supported by the Clinician Scientist program of the Medical Faculty of the University of Tübingen. V.S., L.B., N.B., G.C.B., N.C., G.D.P., G.S.G., V.K., P.M.V., M.T.P., W.P., C.R., W.S., I.S., A.B., K.B., B.K.C., D.G., J.K., C.L., N.Q., and D.R. report no additional disclosures. Angelo Antonini, MD, PhD,22 Amit Batla, MD,23 Kailash Bhatia, MD,23 Barbara K. Changizi, MD,16 Thomas Gasser, MD,24 David Goldstein, MD, PhD,25 Tanya Gurevich, MD,26 Jiri Klempir, MD, PhD,27 Clemence Léung, MD,6 Nikolaus McFarland, MD, PhD,28 Niall Quinn, MD,23 Diego Rodriguez, MD,16 Benjamin Roeben, MD24. 6Department of Clinical Pharmacology and Neurosciences, Clinical Investigation Center CIC1436, Toulouse Parkinson Expert Centre, Toulouse NeuroToul Center of Excellence in Neurodegeneration (COEN), French NS-Park/F-CRIN Network, University of Toulouse 3, CHU of Toulouse, INSERM, Toulouse, France. 16Department of Neurology, Brigham and Women's Hospital and Harvard Medical School, Ann Romney Center for Neurologic Disease, Boston, Massachusetts, USA. 22Parkinson and Movement Disorders Unit, Department of Neuroscience, University of Padua, Padua, Italy. 23UCL Queen Square Institute of Neurology, London, United Kingdom. 24Department of Neurodegeneration, Hertie-Institute for Clinical Brain Research and German Center for Neurodegenerative Diseases, University of Tuebingen, Tuebingen, Germany. 25Clinical Neurocardiology Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA. 26Movement Disorders Unit, Neurological Institute, Tel Aviv Medical Center, 6,423,906 Tel-Aviv, Israel; School of Medicine and Sagol School of Neuroscience, Tel-Aviv University, Tel-Aviv, Israel. 27Charles University and General University Hospital, Prague, Czech Republic. 28Department of Neurology, University of Florida College of Medicine, Gainesville, Florida, USA. The data that supports the findings of this study are available in the supplementary material of this article. Data S1. Supporting Information. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

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