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. 2022 Sep;279(9):4473-4483.
doi: 10.1007/s00405-022-07410-x. Epub 2022 May 5.

Pharyngolaryngeal semiology and prognostic factors in multiple system atrophy

N El Fassi  1 , Y Gallois  2 , S Crestani  2 , P Fichaux-Bourrin  2 , F Ory  3 , M Fabbri  3 , A Pavy le Traon  3 , V Woisard  2
Affiliations

Pharyngolaryngeal semiology and prognostic factors in multiple system atrophy

N El Fassi et al. Eur Arch Otorhinolaryngol. 2022 Sep.

Abstract

Introduction: Multiple system atrophy (MSA) is a rare degenerative neurological disorder in adults. It induces parkinsonian and/or cerebellar syndrome associated with dysautonomia. Pharyngolaryngeal symptoms are common. Our aim is to describe the Pharyngolaryngeal semiology on one hand, and to ascertain whether the presence of these symptoms represents a prognostic factor for MSA on the other.

Methods: Thus, we carried out a retrospective, single-centre study, on a cohort receiving care at the centre of reference for MSA. The patients were referred for otorhinolaryngology assessment. The data was collected over the year 2020 with the help of computer software from the university hospital centre (UHC). Firstly, we described the Pharyngolaryngeal semiology specific to MSA by questioning patients, and by the results of nasofibroscopic examinations and swallowing tests. We then used multivariate analysis of variance to describe the prognostic factors of MSA progression (in UMSARS I and II points per month of progression) and survival (number of years between the first symptoms and death).

Results: This study included a hundred and one patients and made it possible to define a Pharyngolaryngeal semiology profile of MSA, which is: a reduction in laryngeal mobility (primarily vocal cord abduction defects), abnormal movements (particularly at rest or when initiating a movement) and a defect in the protection mechanisms of the upper airways. The swallowing difficulties are moderate and the main mechanisms are delayed pharyngeal swallow and/or an oro-pharyngeal transport defect. In the multivariate analyses, the contributing factors are laryngeal anomalies, modification of solid food to fluid food and nutritional complication.

Conclusion: ENT specialists should pay close attention to problems in the Pharyngolaryngeal dynamic and then consider a neurological cause. They can also itemize the clinical factors that could have a negative effect on the prognosis of the patient with MSA. Indeed, early detection makes it possible to provide care for respiratory and nutritional complications.

Keywords: Abnormal movements of the larynx; Dysphagia; Multiple system atrophy; Prognosis; Vocal fold paralysis.

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Conflict of interest statement

The authors have no conflicts of interest.

Figures

Fig. 1
Fig. 1
Phenotype and Diagnosis of MSA (number)
Fig. 2
Fig. 2
Progression of the UMSARS I + II score (The horizontal black line in the boxplot represents the median, and the red cross the mean)
Fig. 3
Fig. 3
Variations in UMSARS I + II score in function to the laryngeal anomalies
Fig. 4
Fig. 4
Solid food diet in function to disease progression
See this image and copyright information in PMC

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