Objective: To evaluate the predictive value of stridor and of its latency of onset in a cohort of Multiple System Atrophy (MSA) patients referred to a tertiary centre.

Background: MSA is a progressive neurodegenerative disorder with a mean survival ranging from 6.2 to 10 years. Various clinical factors have been reported as predictive of shortened survival, among these stridor has been identified as a negative prognostic indicator in one study but other studies did not confirm these results.

Methods: We retrospectively identified all patients with a final clinical diagnosis of MSA referred to our Department between 1991 and 2015 and evaluated at least once a year during the disease course. Clinical data were collected from medical records and updated at every follow-up visit. Stridor was defined as present when confirmed by a whole night video-polysomnography and categorized as early if presenting within 3 years of disease onset. Survival data were defined based on time to death from the disease onset and calculated using Kaplan-Meier curves. Predictors were identified in a univariate and multivariable COX regression analysis.

Results: A total of 136 MSA patients were included (88 males; 68 MSA with predominant parkinsonism), 113 were deceased at the time of the study. On Kaplan-Meier curve the median duration of illness was 7.84 years. Stridor was diagnosed in 42 (30.88%) patients and 22 of these presented an early stridor onset. The overall survival did not differ between patients with and without stridor (p= 0.3467), while the risk of death in patients with early stridor onset was higher compared to those developing this symptom later (p= 0.0209). The incidence rate of death was 13 per 100 person-year in the first group and 9 per 100 person-year in the second one. In the stridor subgroup, early stridor onset and modality of autonomic onset (orthostatic hypotension vs. urinary dysfunction) predicted unfavourable survival in a univariate analysis. At the multivariable COX model the early stridor onset remained an independent predictor of mortality (HR= 3.21, p= 0.006).

Conclusions: The study showed that the overall survival did not differ between MSA patients with and without stridor, however we demonstrated for the first time that early stridor onset is an independent risk factor for shorter survival.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/early-stridor-onset-predicts-survival-in-multiple-system-atrophy/