PT  - JOURNAL ARTICLE
AU  - Cortelli, Pietro
AU  - Calandra-Buonaura, Giovanna
AU  - Benarroch, Eduardo E.
AU  - Giannini, Giulia
AU  - Iranzo, Alex
AU  - Low, Phillip A.
AU  - Martinelli, Paolo
AU  - Provini, Federica
AU  - Quinn, Niall
AU  - Tolosa, Eduardo
AU  - Wenning, Gregor K.
AU  - Abbruzzese, Giovanni
AU  - Bower, Pamela
AU  - Alfonsi, Enrico
AU  - Ghorayeb, Imad
AU  - Ozawa, Tetsutaro
AU  - Pacchetti, Claudio
AU  - Pozzi, Nicolò Gabriele
AU  - Vicini, Claudio
AU  - Antonini, Angelo
AU  - Bhatia, Kailash P.
AU  - Bonavita, Jacopo
AU  - Kaufmann, Horacio
AU  - Pellecchia, Maria Teresa
AU  - Pizzorni, Nicole
AU  - Schindler, Antonio
AU  - Tison, François
AU  - Vignatelli, Luca
AU  - Meissner, Wassilios G.
TI  - Stridor in multiple system atrophy
AID  - 10.1212/WNL.0000000000008208
DP  - 2019 Oct 01
TA  - Neurology
PG  - 630--639
VI  - 93
IP  - 14
4099  - http://n.neurology.org/content/93/14/630.short
4100  - http://n.neurology.org/content/93/14/630.full
SO  - Neurology2019 Oct 01; 93
AB  - Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a combination of autonomic failure, cerebellar ataxia, and parkinsonism. Laryngeal stridor is an additional feature for MSA diagnosis, showing a high diagnostic positive predictive value, and its early occurrence might contribute to shorten survival. A consensus definition of stridor in MSA is lacking, and disagreement persists about its diagnosis, prognosis, and treatment. An International Consensus Conference among experts with methodological support was convened in Bologna in 2017 to define stridor in MSA and to reach consensus statements for the diagnosis, prognosis, and treatment. Stridor was defined as a strained, high-pitched, harsh respiratory sound, mainly inspiratory, occurring only during sleep or during both sleep and wakefulness, and caused by laryngeal dysfunction leading to narrowing of the rima glottidis. According to the consensus, stridor may be recognized clinically by the physician if present at the time of examination, with the help of a witness, or by listening to an audio recording. Laryngoscopy is suggested to exclude mechanical lesions or functional vocal cord abnormalities related to different neurologic conditions. If the suspicion of stridor needs confirmation, drug-induced sleep endoscopy or video polysomnography may be useful. The impact of stridor on survival and quality of life remains uncertain. Continuous positive airway pressure and tracheostomy are both suggested as symptomatic treatment of stridor, but whether they improve survival is uncertain. Several research gaps emerged involving diagnosis, prognosis, and treatment. Unmet needs for research were identified.CPAP=continuous positive airway pressure; DISE=drug-induced sleep endoscopy; IRCCS-ISNB=Istituto di Ricovero e Cura a Carattere Scientifico delle Scienze Neurologiche di Bologna; MSA=multiple system atrophy; MSA-C=MSA-cerebellar; MSA-P=MSA-parkinsonian; VPSG=video polysomnography