Abstract
BackgroundInflammatory responses in the cerebrospinal fluid (CSF) of patients with sporadic Creutzfeldt-Jakob disease (sCJD) remain elusive.MethodsWe conducted a case-control study, in which 14 patients with sCJD, 14 with noninflammatory neurological disorders, and 14 with autoimmune encephalitis were enrolled. We used the suspension array system to measure the concentrations of 27 cytokines in CSF. The cytokine titers of the three groups were compared, and the correlation between the relevant cytokine titers and clinical parameters was investigated in the patients with sCJD.ResultsLevels of the two cytokines interleukin (IL)-1 receptor antagonist and IL-17 were significantly elevated in the patients with sCJD compared with those in the patients with noninflammatory neurological disorders: IL-17 levels in sCJD were approximately ten times higher than in the noninflammatory neurological disorders (mean, 35.46 vs. 3.45 pg/ml; P < 0.001) but comparable to that in encephalitis (mean, 32.16 pg/ml). In contrast, levels of classical proinflammatory cytokines such as IL-12(p70) and tumor necrosis factor-α were increased only in encephalitis. Although not significant, IL-17 titers tended to be higher in the patients with shorter disease duration before CSF sampling (r = -0.452; P = 0.104) and in those with lower CSF total protein concentrations (r = -0.473; P = 0.086).ConclusionsIL-17 is significantly increased in CSF in sCJD, which can be an early event in the pathogenesis of sCJD.
Highlights
Inflammatory responses in the cerebrospinal fluid (CSF) of patients with sporadic Creutzfeldt-Jakob disease remain elusive
Immunological processes have been poorly recognized in the central nervous system (CNS) of patients with Creutzfeldt-Jakob disease (CJD), partly because pleocytosis or increased protein levels are rarely observed in the cerebrospinal fluid (CSF) of patients with CJD
We systematically measured levels of multiple proinflammatory and anti-inflammatory cytokines in CSF of patients with CJD to detect CNS inflammatory responses that can be associated with the pathogenesis of CJD
Summary
Inflammatory responses in the cerebrospinal fluid (CSF) of patients with sporadic Creutzfeldt-Jakob disease (sCJD) remain elusive. Immunological processes have been poorly recognized in the central nervous system (CNS) of patients with CJD, partly because pleocytosis or increased protein levels are rarely observed in the cerebrospinal fluid (CSF) of patients with CJD. There is still uncertainty regarding cytokine abnormalities, in patients with CJD, because some reports suggest an increase in levels of the proinflammatory cytokines [8,9] while others suggest an increase in levels of the anti-inflammatory cytokines [10,11] in CSF.
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