Objective: To assess motor symptoms and their demographic correlates in MSA patients included in the CMSAR as well their impact on daily living activities.

Background: Motor symptoms are one of the most disabling features in MSA, and do not respond to levodopa or other drugs. The parkinsonian variant (MSA-P) tends to have a worse prognosis with lower survival rate; however this has not always been confirmed in large prospective studies. We have analyzed motor symptoms in our cohort by subtype and other demographic data to see if there were any clear risk factors for worse prognosis.

Methods: We analyzed baseline demographic and clinical data from 69 MSA cases included in the CMSAR. Hoehn & Yahr stages (H&Y), EuroQol-5D and the Unified MSA rating scale (UMSARS) including Global Disability Score (GDS) were applied to assess motor involvement and its impact in quality of life and daily activities.

Results: The cohort includes 69 patients, of which 32 are females. The mean age at basal visit is 63.5 (SD: 8.4) years, and the mean disease duration 5 (SD: 3 years). There are 36 MSA-P, and 33 cerebellar subtype (MSA-C) cases. The mean total UMSARS score was 49.9(r= 16 to 91) and 49.2% of the cases were H&Y stage >4. MSA-P variants had higher UMSARS score (p=0.004), specifically for bradykinesia, rigidity, rest tremor and posture as would be expected, but also had more difficulties with daily tasks (eating, dressing and hygiene)(p<0.05) and higher GDS scores (p=0.002). Gender differences were significant for females scoring higher on gait impairment (p=0.013), impaired postural reflexes (p=0.002), and probability of falls (p<0.0001). Females also had higher H&Y stage (p=0.002) and GDS (p=0.003) with worse Euro-QoL (p=0.008). Younger age at onset cases had lower score on UMSARS (p= 0.009) with similar disease duration. There were no significant differences between gender or MSA phenotype for disease duration, age at basal visit or age at disease onset.

Conclusions: In this cohort MSA-P variants are more disabled and have more difficulties to fulfill daily tasks. Females also seem to have a more aggressive disease with more postural instability and gait impairment, as well as higher GDS scores. In contrast, younger age at onset predicts a slightly better prognosis; however disease duration is the most predictive factor for worse motor outcome.

References: 1. Starhof C, Korbo L, Funch C, Winge K and Friis S. Clinical Features in a Danish Population-Based Cohort of Probable Multiple System Atrophy Patients. Neuroepidemiology 2016;46:261–267. DOI: 10.1159/000444325. 2. Wenning GK, Geser F, Krismer F, Seppi K, Duerr S, Boesch S, Köllensperger M, Goebel G, Pfeiffer KP, Barone P, Pellecchia MT, Quinn NP, Koukouni V, Fowler CJ, Schrag A, Mathias CJ, Giladi N, Gurevich T, Dupont E, Ostergaard K, Nilsson CF, Widner H, Oertel W, Eggert KM, Albanese A, del Sorbo F, Tolosa E, Cardozo A, Deuschl G, Hellriegel H, et al: The natural history of multiple system atrophy: a prospective European cohort study. Lancet Neurol 2013, 12:264–74.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-catalonian-multiple-system-atrophy-registry-cmsar-the-motor-features-and-their-impact-on-disability-status-in-msa-patients/