Objective: To define a clinical biomarker that allows for early identification of Multiple System Atrophy.

Background: In its early stages MSA can be difficult to differentiate from idiopathic Parkinson’s disease. Emphasis has been put on identifying premotor symptoms to allow for an early diagnosis. The occurrence of vegetative symptoms in addition to motor impairment enables the clinical diagnosis in the advanced stages of MSA. Laryngeal abnormalities become clinically evident in late MSA stages and can manifest e.g. in laryngeal stridor. Before the clinical manifestation of laryngeal dysfunction, abnormal VF motion can already be seen in patients that not yet fulfill the diagnostic criteria of MSA.

Method: 8 MSA patients according to the Gilman criteria underwent flexible endoscopic evaluation of the swallowing (FEES). To assess MSA specific impairment of the laryngopharynx, patients underwent the FEES-MSA-protocol allowing for a standardized endoscopic evaluation of the pharyngolaryngeal and swallowing function.

Results: 8 MSA patients (4 female) underwent the FEES-MSA-protocol (7 MSA-P, 1 MSA-C). Mean age was 59.9 ± 6.9 years, mean disease duration 3.0 ± 1.2 years. 2 patients showed diurnal inspiratory laryngeal stridor as a clinical sign of laryngeal pathology. During FEES, all patients showed irregular arytenoid cartilage movements (ACM), 6 at rest and 2  during provoking maneuvers. All patients had glottis opening restriction. 3 patients had VF fixation in the paramedian position, 1 showed paradoxical VF motion.5 patients had piecemeal deglutition for all consistencies. 7 showed premature spillage. All patients showed pharyngeal phase dysfunction. 6 patients had pharyngeal residues in the valleculae. 5 patients showed penetration/ aspiration events. Characteristics of pharyngolaryngeal findings did not differ between the MSA sub-types.

Conclusion: Pharyngolaryngeal abnormalities have a high prevalence in MSA. Our findings suggest that characteristic pathologic findings can be revealed on flexible endoscopy before becoming evident clinically. Irregular arytenoid cartilages movement (ACM) at rest or with provoking maneuvers might serve as a diagnostic clinical biomarker and facilitate early identification of MSA. We strongly suggest that all patients with suspected MSA should undergo FEES using a structured FEES-MSA-protocol to look for disease specific findings that support the diagnosis and allow for its earl identification.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/shaking-palsy-of-the-larynx-a-potential-biomarker-for-multiple-system-atrophy/