Objective: Our aim was to explore the predictors of survival in Chinese multiple system atrophy (MSA) patients.

Background: MSA is a rare, fatal neurodegenerative disorder with symptoms of autonomic failure plus parkinsonism, cerebellar ataxia, or both. Predictors of survival of MSA remain largely unknown.

Methods: A total of 339 probable MSA patients were enrolled. Disease severity was assessed by the Unified Multiple System Atrophy Rating Scale (UMSARS). Kaplan-Meier curves were used to compare survival time. Cox proportional hazards models were used to calculate hazard ratios for shorter survival using age of disease onset, sex, clinical phenotype, neurological and autonomic manifestations as categorical variables.

Results: Among the total of patients, 195 patients were MSA-C subtype and 144 were MSA-P subtype. At baseline, there were no significant differences in the disease duration, the frequency of autonomic symptom as initial symptom and autonomic symptoms between the MSA-P and MSA-C patients, however, the MSA-P patients showed higher UMSARS-I, UMSARS-II, and UMSARS-IV scores than the MSA-C patients. Twelve (3.52%) patients were lost during follow-up, 118 patients were deceased, and 209 patients (follow-up period from 12 to 82 months) were alive at the time of the last visit. Median survival from symptom onset to death was 7.3 years (95% CI=6.8–7.8) and median survival from enrollment to death was 4.5 years (3.7–4.6) according to Kaplan-Meier analysis. The older age of onset (>57 years) showed shorter survival time (p=0.027). There were no differences in survival time regarding sub-type or sex. The adjusted Cox proportional hazard model retained the following unfavorable predictors of survival: (i) autonomic onset (hazard ratio=1.736, p=0.029); (ii) UMSARS-IV > 2 (hazard ratio=1.944, p=0.001); (iii) orthostatic hypotension (hazard ratio=1.923, p=0.003); (iv) falls within 3 years (hazard ratio=1.570, p=0.029); (v) age of onset > 57 (hazard ratio=1.623, p=0.011).

Conclusions: Survival time of Chinese MSA patients was similar with that of Caucasian population. Our study suggests that autonomic onset, orthostatic hypotension, older age onset and falls within 3 years are potential predictors for survival of MSA patients. Additionally, severe global disability measured by UMSARS-IV is the first time suggested to be a poor survival predictor for MSA.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-predictor-factors-for-survival-in-chinese-multiple-system-atrophy-patients/