| Objective: Multiple system atrophy(MSA) is a rare, sporadic and progressive neurodegenerative disorder which involves multiple functional or anatomical regions of the central nervous system with unknown etiology, presenting with motor and non-motor symptoms. There is no specific treatment for the disorder and its prognosis is poor. Most of the patients with this disorder have impaired motor activity in late stage of the disorder. Furthermore, the prognostic factors of MSA have not been well characterized because of controversial results in previous studies. This study was to analyze the clinical features of MSA patients and to investigate the predictive factors affecting the prognosis of MSA.Methods: 84 patients with probable MSA admitted to two different hospitals, from March 2013 to March 2015, were included in this study(Gilman et al in 2008). All cases were interviewed and examined by two neurologists who had expertise in movement disorder. Study duration was 2 years with follow-ups every 6 months. 9 possible prognostic factors were screened and quantified. Data were analyzed with SPSS 22.0(IBM SPSS Statistics). Survival was analyzed using Kaplan-Meier plots. Univariate analysis was performed through log-rank test between subgroups. Covariates were analyzed with Cox proportional hazards models regression analysis. P < 0.05 was considered statistically significant.Results: 51 patients were men and 33 were women with a sex radio of 1.55:1(M: F). 49 cases were diagnosed with MSA-C and 35 with MSA-P(C: P=1.4:1). All the patients had involvement of autonomic failure: 72.6% with urinary or fecal incontinence, 75.0% with orthostatic hypotension with or without syncope, and 47.6% with both urinary or fecal incontinence and orthostatic hypotension. 98.0% of the male patients presented with erectile dysfunction. Regarding the involvement of the 4 systems(autonomic, pyramidal, extrapyramidal and cerebellar system), 58.3% had 3 systems impaired, 22.6% had 2 systems and 19.0% had 4 system involvement. 58.3% of the patients had sweating dysfunction while 42.9% had skin temperature paresthesia. 73.8% of the patients developed non-motor initial symptoms compared with 26.2% having motor symptom. The median interval between disease onset to combined motor and autonomic involvement was 24 months. Up to the end of the study, 54.8% of patients were unable to walk independently, 33.3% required aid in walking, while 13.1% were wheelchair bound, 7.1% bed bound, and 1.9% dead. The median survival time from disease onset to aid-requiring walking state was 68 months. Univariate analysis showed that initial symptom, age of onset, the interval between disease onset to combined motor and autonomic involvement were important predicting factors for the inability to walk independently(P<0.05). COX regression analysis demonstrated that older than 52 at onset and initial motor involvement had significantly higher risks of being unable to walk independently.Conclusion: Clinical features of MSA are characterized by salient autonomic dysfunction as well as involvement of multiple motor systems. Autonomic dysfunction includes not only significant urinary and fecal incontinence and orthostatic hypotension, but also sweating dysfunction and skin temperature paresthesia in a goodly number of patients. Besides, most of MSA patients(73.8%) presented initially with non-motor symptoms, higher than previous reports. Late onset(older than 52) and initial motor involvement are predictive factors for the inability to walk independently. |
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