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Stridor in multiple system atrophy: Consensus statement on diagnosis, prognosis, and treatment
Pietro Cortelli1, Giovanna Calandra-Buonaura2, Eduardo E Benarroch2
1From the IRCCS (P.C., G.C.-B., G.G., F.P., L.V.), Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy; Dipartimento di Scienze Biomediche e Neuromotorie (P.C., G.C.-B., G.G., P.M., F.P.), Università di Bologna, Bologna, Italy; Department of Neurology (E.E.B., P.A.L.), Mayo Clinic, Rochester, MN; Multidisciplinary Sleep Unit (A.I.), Neurology Service, Hospital Clinic de Barcelona, IDIBAPS CIBERNED, Barcelona, Spain; UCL Queen Square Institute of Neurology (N.Q.), Queen Square, London; Parkinson's Disease and Movement Disorders Unit (E.T.), Neurology Service, Hospital Clinic de Barcelona; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS) (E.T.), University of Barcelona (UB), and Centre for Networked Biomedical Research on Neurodegenerative Diseases (CIBERNED), Barcelona, Spain; Department of Neurology (G.K.W.), Innsbruck Medical University, Innsbruck, Austria; Department of Neuroscience (G.A.), Rehabilitation, Ophthalmology, Genetics and Maternal Child Health, University of Genoa, Genoa, Italy; The Multiple System Atrophy Coalition, Inc. (P.B.), Charlotte, NC; Neurophysiopathology Unit (E.A.), IRCCS "C. Mondino" Foundation, Pavia, Italy; Department of Clinical Neurophysiology (I.G.), CHU de Bordeaux, Bordeaux, France; Université de Bordeaux (I.G.), Institut de Neurosciences Cognitives et Intégratives d'Aquitaine, Bordeaux, France; CNRS (I.G.), Institut de Neurosciences Cognitives et Intégratives d'Aquitaine, Bordeaux, France; Department of Neurology (T.O.), Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Minami Uonuma, Niigata, Japan; Parkinson's Disease and Movement Disorders Unit (C.P., N.G.P.), IRCCS "C. Mondino" Foundation, Pavia; Dipartimento di Medicina Specialistica (C.V.), Diagnostica e Sperimentale (DIMES), University of Bologna, Bologna, Italy; Dipartimento di Scienze biomediche e chirurgico specialistiche (C.V.), University of Ferrara, Ferrara, Italy; Department of Neurosciences (A.A.), University of Padua, Padua, Italy; Department of Clinical and Motor Neuroscience (A.P.B.), UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London; Spinal Unit (J.B.), Montecatone Rehabilitation Institute, Imola, Italy; Department of Neurology (H.K.), New York University School of Medicine, New York, NY; Department of Medicine (M.T.P.), Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Salerno, Italy; "Luigi Sacco" Department of Biomedical and Clinical Sciences (N.P., A.S.), University of Milan, Milan, Italy; Service de Neurologie (F.T., W.G.M.), CRMR Atrophie Multisystématisée, CHU Bordeaux, Bordeaux, France; Univ. de Bordeaux (F.T., W.G.M.), Institut des Maladies Neurodégénératives, Bordeaux, France; and Department Medicine (W.G.M.), University of Otago, Christchurch, and New Zealand Brain Research Institute, Christchurch, New Zealand. pietro.cortelli@unibo.it.
Multiple system atrophy (MSA) experts defined laryngeal stridor, a key diagnostic sign. Consensus statements address stridor
Area of Science:
- Neurology
- Otolaryngology
- Respiratory Medicine
Background:
- Multiple system atrophy (MSA) is a neurodegenerative disease with autonomic failure, ataxia, and parkinsonism.
- Laryngeal stridor is a significant diagnostic feature in MSA, potentially impacting survival.
- Lack of consensus on stridor definition, diagnosis, prognosis, and treatment hinders patient care.
Purpose of the Study:
- To establish a consensus definition for laryngeal stridor in MSA.
- To develop consensus statements on the diagnosis, prognosis, and treatment of stridor in MSA.
- To identify research gaps and unmet needs concerning MSA-related stridor.
Main Methods:
- An International Consensus Conference convened experts in 2017.
- Methodological support was provided to guide the conference.
- Consensus statements were developed through expert deliberation.
Main Results:
- Stridor defined as a strained, high-pitched, harsh respiratory sound due to laryngeal dysfunction, occurring during sleep or wakefulness.
- Clinical recognition via physician observation, witness accounts, or audio recordings.
- Laryngoscopy, drug-induced sleep endoscopy, and polysomnography suggested for diagnosis and exclusion of other conditions.
Conclusions:
- Consensus definition and diagnostic approaches for MSA-related stridor established.
- Prognostic impact and optimal treatment strategies for stridor in MSA require further investigation.
- Significant research gaps identified, highlighting unmet needs in MSA stridor management.
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