Early strong intrathecal inflammation in cerebellar type multiple system atrophy by cerebrospinal fluid cytokine/chemokine profiles: A case control study

Background: the pathology of multiple system atrophy cerebellar-type (msa-c) includes glial inflammation; however,cerebrospinal fluid (csf) inflammatory cytokine profiles have not been investigated. in this study,we determined csf cytokine/chemokine/growth factor profiles in msa-c and compared them with those in hereditary spinocerebellar ataxia (sca). methods: we collected clinical data and csf from 20 msa-c patients,12 hereditary sca patients,and 15 patients with other non-inflammatory neurological diseases (ond),and measured 27 cytokines/chemokines/growth factors using a multiplexed fluorescent bead-based immunoassay. the size of each part of the hindbrain and hot cross bun sign (hcbs) in the pons was studied by magnetic resonance imaging. results: granulocyte-macrophage colony-stimulating factor (gm-csf),interleukin (il)-6,il-7,il-12,and il-13 levels were significantly higher in msa-c and sca compared with ond. in msa-c,il-5,il-6,il-9,il-12,il-13,platelet-derived growth factor-bb,macrophage inflammatory protein (mip)-1α,and gm-csf levels positively correlated with anteroposterior diameters of the pontine base,vermis,or medulla oblongata. by contrast,in sca patients,il-12 and mip-1α showed significant negative correlations with anteroposterior diameters of the pontine base,and unlike msa-c,there was no cytokine with a positive correlation in sca. il-6 was significantly higher in msa-c patients with the lowest grade of hcbs compared with those with the highest grade. macrophage chemoattractant protein-1 (mcp-1) had a significant negative correlation with disease duration only in msa-c patients. tumor necrosis factor-alpha,il-2,il-15,il-4,il-5,il-10,and il-8 were all significantly lower in msa-c and sca compared with ond,while il-1ra,an anti-inflammatory cytokine,was elevated only in msa-c. il-1β and il-8 had positive correlations with unified multiple system atrophy rating scale part 1 and 2,respectively,in msa-c. conclusions: although csf cytokine/chemokine/growth factor profiles were similar between msa-c and sca,pro-inflammatory cytokines,such as il-6,gm-csf,and mcp-1,correlated with the disease stage in a way higher at the beginning only in msa-c,reflecting early stronger intrathecal inflammation. © 2017 the author(s).