Dysphagia in multiple system atrophy consensus statement on diagnosis, prognosis and treatment
Parkinsonism & Related Disorders. 2021-05-01; 86: 124-132
DOI: 10.1016/j.parkreldis.2021.03.027
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Calandra-Buonaura G(1), Alfonsi E(2), Vignatelli L(3), Benarroch EE(4), Giannini
G(5), Iranzo A(6), Low PA(7), Martinelli P(8), Provini F(9), Quinn N(10), Tolosa
E(11), Wenning GK(12), Abbruzzese G(13), Bower P(14), Antonini A(15), Bhatia
KP(16), Bonavita J(17), Pellecchia MT(18), Pizzorni N(19), Tison F(20), Ghorayeb
I(21), Meissner WG(22), Ozawa T(23), Pacchetti C(24), Pozzi NG(25), Vicini C(26),
Schindler A(27), Cortelli P(28), Kaufmann H(29).
Author information:
(1)IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy;
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna,
Bologna, Italy. Electronic address: .
(2)Neurophysiopathology Unit, IRCCS Mondino Foundation, Pavia, Italy. Electronic
address: .
(3)IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy.
Electronic address: .
(4)Department of Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address:
.
(5)IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy;
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna,
Bologna, Italy. Electronic address: .
(6)Multidisciplinary Sleep Unit, Neurology Service, Hospital Clinic de Barcelona,
IDIBAPS CIBERNED, Barcelona, Spain. Electronic address: .
(7)Department of Neurology, Mayo Clinic, Rochester, MN, USA. Electronic address:
.
(8)Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna,
Bologna, Italy. Electronic address: .
(9)IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy;
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna,
Bologna, Italy. Electronic address: .
(10)UCL Queen Square Institute of Neurology, Queen Square, London, UK. Electronic
address: .
(11)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: .
(12)Department of Neurology, Innsbruck Medical University, Innsbruck, Austria.
Electronic address: .
(13)Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics and
Maternal Child Health, University of Genoa, Genoa, Italy. Electronic address:
.
(14)The Multiple System Atrophy Coalition, Inc, Charlotte, NC, USA. Electronic
address: .
(15)Department of Neurosciences, University of Padua, Padua, Italy. Electronic
address: .
(16)Department of Clinical and Motor Neuroscience, UCL Institute of Neurology,
National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Electronic address: .
(17)Villa Rosa Rehabilitation Department, Pergine Valsugana, Azienda Provinciale
Servizi Sanitari Trento, Italy. Electronic address: .
(18)Department of Medicine, Surgery and Dentistry « Scuola Medica Salernitana »,
University of Salerno, Salerno, Italy. Electronic address: .
(19) »Luigi Sacco » Department of Biomedical and Clinical Sciences, University of
Milan, Milan, Italy. Electronic address: .
(20)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: .
(21)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: .
(22)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:
.
(23)Department of Neurology, Uonuma Institute of Community Medicine, Niigata
University Medical and Dental Hospital, 4132 Urasa, Minami Uonuma, Niigata,
949-7302, Japan. Electronic address: .
(24)Parkinson’s Disease and Movement Disorders Unit, IRCCS Mondino Foundation,
Pavia, Italy. Electronic address: .
(25)Parkinson’s Disease and Movement Disorders Unit, IRCCS Mondino Foundation,
Pavia, Italy. Electronic address: .
(26)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: .
(27) »Luigi Sacco » Department of Biomedical and Clinical Sciences, University of
Milan, Milan, Italy. Electronic address: .
(28)IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy;
Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna,
Bologna, Italy. Electronic address: .
(29)Department of Neurology, New York University School of Medicine, New York,
NY, USA. Electronic address: .
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.
Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.
DOI: 10.1016/j.parkreldis.2021.03.027
PMID: 33839029