To investigate the intra- and extracellular levels of various
cytokines and
chemokines in CSF in chronic inflammatory demyelinating
polyneuropathy (
CIDP) and vasculitic neuropathy (VN), 16
cytokines, IL-1beta,
IL-2,
IL-4,
IL-5,
IL-6,
IL-7,
IL-8,
IL-10,
IL-12 (p70),
IL-13,
IL-17, IFN-gamma,
TNF-alpha,
G-CSF, MCP-1 and
MIP-1beta, were measured in CSF supernatant by a multiplexed fluorescent bead-based immunoassay and intracellular production of IFN-gamma and
IL-4 in CSF CD4+ T cells were simultaneously measured by flow cytometry in 14 patients with
CIDP, 8 patients with VN and 25 patients with other noninflammatory neurologic diseases (OND). In the CSF supernatant, a significant increase of
IL-17,
IL-8 and
IL-6, and a significant decrease of
IL-4,
IL-5 and
IL-7 levels were detected in pretreated
CIDP as compared with OND. A significant increase of
IL-6,
IL-8 and
IL-10 levels was found in pretreated VN. Both
IL-17 and
IL-8 levels correlated strongly with CSF
protein levels in
CIDP, although the correlation of
IL-6 levels was weak. In CSF CD4+ T cells, IFN-gamma+ IL-4- cell percentages were markedly elevated in
CIDP compared with OND, but not in VN, resulting in a significant increase of intracellular IFN-gamma/IL-4 ratio in
CIDP, even in the absence of CSF
pleocytosis. The nonresponders to
intravenous immunoglobulins (IVIGs) showed a significantly lower IFN-gamma- IL-4+ CD4+ T cell percentage, and tended to have a higher intracellular IFN-gamma/IL-4 ratio than the responders in CSF. Marked upregulation of Th1
cytokine,
IL-17, and downregulation of Th2
cytokines, together with infiltration of IFN-gamma-producing CD4+ T cells are useful markers for
CIDP, while several Th2
cytokines are upregulated in VN in CSF.