![]() For example, one large study showed higher rates of preexisting autoimmune disease in patients with ALS, including multiple sclerosis, polymyositis, and Sjögren’s syndrome, when compared with expected population-based numbers. Some neurodegenerative disorders, including variants of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS), have been associated with various systemic autoimmune disorders ( 1, 2). There is growing evidence for a causal relationship between neurodegeneration and neuroinflammation. The Michigan Brain Bank is supported in part through NIH grant P30AG053760. These findings together support the hypothesis that systemic autoimmune disease plays a role in neurodegeneration.įUNDING. The 2 cases we describe of autopsy-proven MSA support that MSA pathology can explain neurologic disease in a subset of patients with pSS. Our findings provide evidence for an association between MSA and pSS that is specific to both pSS, among autoimmune disorders, and MSA, among neurodegenerative disorders. The 2 patients we described with pSS and progressive neurodegenerative disease showed classic MSA pathology at autopsy.ĬONCLUSION. Our cohort of 26 autopsy-proven patients with MSA had a significantly higher prevalence of pSS compared with a cohort of 115 patients with other autopsy-proven neurodegenerative disorders (8% vs. 0%, P = 0.02) compared with 776 patients in a control cohort of patients with other autoimmune disorders. Our cohort of 308 patients with pSS had a greater incidence of MSA compared with 4 large population-based studies and had a significantly higher prevalence of at least probable MSA (1% vs. We additionally reviewed the neuropathologic findings in 2 patients with pSS, cerebellar degeneration, parkinsonism, and autonomic dysfunction. These data were compared with rates in respective control groups. We performed a retrospective cohort study assessing (a) rates of MSA in a cohort of patients with pSS and (b) rates of pSS in a cohort of patients with MSA. Our objective was to investigate whether primary Sjögren’s syndrome (pSS) is associated with multiple system atrophy (MSA).
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