Objective: To characterize the clinical presentation, to identify markers of disease progression and to assess diagnostic utility of ‘red flags’ in subjects with multiple system atrophy (MSA) through collecting basic demographic and disease-specific patient information.

Background: MSA is a rare progressive neurodegenerative disorder characterized by Parkinsonian, cerebellar, pyramidal and autonomic features in any combination. The influence of clinical features such as subtype, onset of disease including initial symptoms and age as well as gender and early autonomic dysfunction on disease progression and survival are still controversial.

Methods: In this retrospective study we analysed the medical history of all patients with clinically possible or probable MSA who were treated at the movement disorder unit of Innsbruck between January 1995 and September 2015. Descriptive statistics of nominal and ordinal variables were performed and appropriate parametric or non-parametric tests were applied. Furthermore, Kaplan-Meier analysis for calculation of survival and Cox regression model for identification of predictors were used.

Results: So far 71 out of a total of 170 MSA patients have been analysed; 11 patients fulfilled criteria for possible and 60 for probable MSA. The predominant subtype of MSA was the Parkinson variant (63%), 55% were male. Average age of symptom onset was 57.7 years. In 54% motor symptoms were present at symptom onset followed by a mixed presentation of motor and autonomic dysfunction in 31% and an isolated dysautonomic onset in 15%. Mean disease duration from symptom onset to diagnosis was 46.0 months. Twenty-six patients had a beneficial L-Dopa response, which was relatively short with a mean duration of 18.3 months. Average time (in months) of reaching disability milestones after onset of the initial feature was 49.9 (falls at least once a month), 65.8 (care dependency), 73.2 (wheel chair bound) and 83.0 (bedridden state).

Conclusions: This retrospective study will help identify prognostic markers by systematically collecting clinical features throughout the disease course.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/innsbruck-multiple-system-atrophy-cohort-study-an-interim-analysis/