Abstract
The objective of this study was to explore the causes of death in Chinese patients with multiple system atrophy (MSA) as well as differences in the cause of death according to sex, subtype, disease onset, and whether the disease was accompanied by nocturnal stridor. A total of 131 MSA patients were enrolled and followed up once every year until their deaths. Clinical information was collected by neurologists, and the cause of death of the MSA patients was obtained from the patients’ relatives or caregivers. The current study included 62 MSA with predominant parkinsonism (MSA-P) and 69 MSA with predominant cerebellar ataxia (MSA-C) patients. Median survival time from disease onset to death of the MSA patients was 5.59 years. The most common cause of death was respiratory infection (65.6%). The second most common cause of death was sudden death (14.5%). Other causes included nutritional disorder due to dysphagia (9.2%), urinary tract infection (3.1%), suicide (2.3%), choking (1.5%), cerebrovascular accident (1.5%), myocardial infarction (1.5%), and lymphoma (0.8%). We found that sudden death was more likely to occur in patients with nocturnal stridor than in those without (P<0.001). There were no significant differences in the cause of death according to subtype, sex, or onset symptoms (autonomic failure or motor symptoms). Sudden death is a relatively common cause of death in MSA patients, second only to respiratory infection, especially in patients with nocturnal stridor. The information provided by our study may help to provide better medical care to MSA patients.
Highlights
Multiple system atrophy (MSA) is a sporadic, adult-onset neurodegenerative disease with a heterogeneous combination of autonomic failure, cerebellar ataxia, parkinsonian features, and pyramidal signs [1]
The second most common cause of death was sudden death (19, 14.5%), which occurred in 12 patients (9.2%) during the day and in 7 patients (5.3%) at night
There were no significant differences in the causes of death between the MSA with predominant parkinsonism (MSA-P) and MSA with predominant cerebellar ataxia (MSA-C) patients (Table 2), between the male and female patients (Table 2), or between the patients with autonomic failure or motor symptoms as onset symptoms (Table 3)
Summary
Multiple system atrophy (MSA) is a sporadic, adult-onset neurodegenerative disease with a heterogeneous combination of autonomic failure, cerebellar ataxia, parkinsonian features, and pyramidal signs [1]. Varied factors have been presented to predict the survival of patients with MSA, such as autonomic failure, the parkinsonian variant of MSA, and older age of onset [8,9,10]. Patients with MSA have been reported to die of respiratory infection, sudden death, choking, cancer, suicide, and stroke, among other causes [5, 11,12,13,14,15,16,17,18]. The frequency of different causes of death in MSA is inconsistent across studies. The sample sizes of these studies that focused on the causes of death in MSA were relatively small. Whether the causes of death in MSA patients differ according to sex, subtypes, and onset symptoms remains largely unknown.
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