Objective: To evaluate clinical and instrumental different features in two case series of multiple system atrophy (MSA) patients with or without laryngeal stridor.

Background: MSA is a neurodegenerative disease involving the central and autonomic nervous system, often leading to severe breathing disorders like stridor. The relationship of this sign with other clinical and instrumental features has not yet been well recognized. The increased activity of laryngeal adductors during inspiration also suggests an abnormal premotor control by the brainstem respiratory networks. Supratentorial and infratentorial magnetic resonance imaging (MRI) patterns were described in MSA.

Methods: We undertook a retrospective study of 60 MSA patients (45 with subtype P and 15 with subtype C) admitted to our center. The subjects were divided into two groups: with (group1) and without stridor (group 2). Video-polysomnography revealed stridor in 19 (32%) patients. All subjects underwent clinical and extensive instrumental examination.

Results: Group 1 and group 2 don’t differ significantly about age at onset, disease duration and severity of motor impairment (UMSARS part II). Compared to group 2, group 1 has more clinical and instrumental global deficits. In fact, it has more prevalence of antecollis (32% vs 22%), RBD (95% vs 81%), cognitive impairment (77% vs 72%), orthostatic hypotension (57% vs 50%), arterial hypotension during the continuous pressure monitoring (64% vs 60%). Dysphagia is described in all the patients with stridor and in 88% of the patients without stridor. In group 1 dysphagia anticipates the stridor onset. The fibroendoscopic evaluation of swallowing shows overlapping findings, while laryngeal and swallowing electromyography displays more frequent and severe alterations in group 1. Group 1 has less supratentorial (56% vs 67%) and more infratentorial (50% vs 36%) MRI alterations.

Conclusions: MSA with stridor may be a partly different form associated to a more diffuse and important infratentorial neuronal loss, in particular in the brainstem, with an impairment of the swallowing mechanisms before the onset of the stridor and more severe clinical course. The presence of stridor in MSA may be considered a marker of disease progression regardless of disease duration and motor severity. Careful and early assessment of the stridor onset can therefore improve the management of the most complex and critical stages of the disease.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-and-instrumental-features-of-multiple-system-atrophy-with-laryngeal-stridor/