Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment.

Angelo Antonini, Horacio Kaufmann, Pietro Cortelli, Antonio Schindler, Claudio Vicini, Nicolò Gabriele Pozzi, Claudio Pacchetti, Tetsutaro Ozawa, Wassilios G Meissner, Imad Ghorayeb, François Tison, Nicole Pizzorni, Maria Teresa Pellecchia, Jacopo Bonavita, Kailash P Bhatia, Giovanna Calandra-Buonaura, Pamela Bower, Giovanni Abbruzzese, Gregor K Wenning, Eduardo Tolosa, Niall Quinn, Federica Provini, Paolo Martinelli, Phillip A Low, Alex Iranzo, Giulia Giannini, Eduardo E Benarroch, Luca Vignatelli, Enrico Alfonsi

Journal: Parkinsonism & related disorders 2022;86():124-132

PMID: 33839029

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Abstract

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure plus cerebellar syndrome and/or parkinsonism. Dysphagia is a frequent and disabling symptom in MSA and its occurrence within 5 years of motor onset is an additional diagnostic feature. Dysphagia can lead to aspiration pneumonia, a recognized cause of death in MSA. Guidelines for diagnosis and management of dysphagia in MSA are lacking. An International Consensus Conference among experts with methodological support was convened in Bologna to reach consensus statements for the diagnosis, prognosis, and treatment of dysphagia in MSA. Abnormalities of the oral and pharyngeal phases of swallowing, esophageal dysfunction and aspiration occur in MSA and worsen as the disease progresses. According to the consensus, dysphagia should be investigated through available screening questionnaires and clinical and instrumental assessment (videofluoroscopic study or fiberoptic endoscopic evaluation of swallowing and manometry) at the time of MSA diagnosis and periodically thereafter. There is evidence that dysphagia is associated with poor survival in MSA, however effective treatments for dysphagia are lacking. Compensatory strategies like diet modification, swallowing maneuvers and head postures should be applied and botulinum toxin injection may be effective in specific conditions. Percutaneous endoscopic gastrostomy may be performed when there is a severe risk of malnutrition and pulmonary complications, but its impact on survival is undetermined. Several research gaps and unmet needs for research involving diagnosis, prognosis, and treatment were identified.

Address: IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy. Electronic address: [email protected].; Neurophysiopathology Unit, IRCCS Mondino Foundation, Pavia, Italy. Electronic address: [email protected].; IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy. Electronic address: [email protected].; Department of Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address: [email protected].; IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy. Electronic address: [email protected].; Multidisciplinary Sleep Unit, Neurology Service, Hospital Clinic de Barcelona, IDIBAPS CIBERNED, Barcelona, Spain. Electronic address: [email protected].; Department of Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address: [email protected].; Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy. Electronic address: [email protected].; IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy. Electronic address: [email protected].; UCL Queen Square Institute of Neurology, Queen Square, London, UK. Electronic address: [email protected].; Parkinson's Disease and Movement Disorders Unit, Neurology Service, Hospital Clinic de Barcelona, Institut D'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona (UB), and Centre for Networked Biomedical Research on Neurodegenerative Diseases (CIBERNED), Barcelona, Spain. Electronic address: [email protected].; Department of Neurology, Innsbruck Medical University, Innsbruck, Austria. Electronic address: [email protected].; Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics and Maternal Child Health, University of Genoa, Genoa, Italy. Electronic address: [email protected].; The Multiple System Atrophy Coalition, Inc, Charlotte, NC, USA. Electronic address: [email protected].; Department of Neurosciences, University of Padua, Padua, Italy. Electronic address: [email protected].; Department of Clinical and Motor Neuroscience, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK. Electronic address: [email protected].; Villa Rosa Rehabilitation Department, Pergine Valsugana, Azienda Provinciale Servizi Sanitari Trento, Italy. Electronic address: [email protected].; Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy. Electronic address: [email protected].; "Luigi Sacco" Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy. Electronic address: [email protected].; Service de Neurologie - Maladies Neurodégénératives, CRMR Atrophie Multisystématisée, CHU Bordeaux, 33000, Bordeaux, France; Univ. de Bordeaux, Institut des Maladies Neurodégénératives, CNRS UMR 5293, 33000, Bordeaux, France. Electronic address: [email protected].; Departement of Clinical Neurophysiology, CHU de Bordeaux, F-33076, Bordeaux, France; Université de Bordeaux, Institut de Neurosciences Cognitives et Intégratives D'Aquitaine, UMR 5287, F-33076, Bordeaux, France; CNRS, Institut de Neurosciences Cognitives et Intégratives D'Aquitaine, UMR 5287, F-33076, Bordeaux, France. Electronic address: [email protected].; Service de Neurologie - Maladies Neurodégénératives, CRMR Atrophie Multisystématisée, CHU Bordeaux, 33000, Bordeaux, France; Univ. de Bordeaux, Institut des Maladies Neurodégénératives, CNRS UMR 5293, 33000, Bordeaux, France; Department Medicine, University of Otago, Christchurch, And New Zealand Brain Research Institute, Christchurch, New Zealand. Electronic address: [email protected].; Department of Neurology, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, 4132 Urasa, Minami Uonuma, Niigata, 949-7302, Japan. Electronic address: [email protected].; Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy. Electronic address: [email protected].; Parkinson's Disease and Movement Disorders Unit, IRCCS Mondino Foundation, Pavia, Italy. Electronic address: [email protected].; Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale (DIMES), University of Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Chirurgico Specialistiche, University of Ferrara, Ferrara, Italy. Electronic address: [email protected].; "Luigi Sacco" Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy. Electronic address: [email protected].; IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy. Electronic address: [email protected].; Department of Neurology, New York University School of Medicine, New York, NY, USA. Electronic address: [email protected].

DOI: 10.1016/j.parkreldis.2021.03.027

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MeSH Terms: Deglutition Disorders, Humans, Multiple System Atrophy