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CXCL13 Or Anti-N-methyl-D-aspartate Receptor Encephalitis Markers
Sep 01, 2017

Anti N- methyl -D- aspartate receptor (NMDAR) encephalitis is an immune mediated disease, mainly affects women and children, and has prominent neuropsychiatric symptoms, such as mental and behavioral abnormalities, reduced speech, seizures, abnormal limb movement etc.. Studies have shown that NMDAR antibodies bind to and internalize receptors, thereby reducing NMDAR function. Although anti NMDAR encephalitis is more serious, it can still be treated. One of the problems, however, is the lack of biomarkers related to disease activity to guide disease treatment or identification of recurrences.

CXCL13 is a B lymphocyte chemotactic factor (CXCL13) that is elevated in autoimmune diseases such as multiple sclerosis and optic myelitis. Dr. Frank and other researchers from the University of Barcelona, detection of anti NMDAR encephalitis patients serum and cerebrospinal fluid (CSF) in the CXCL13 level; and to further explore whether CXCL13 as an effective marker to guide the treatment and prognosis of the disease. Specific results were published in the Journal JAMA Neurol recently.

The study included 167 patients who had been diagnosed with NMDAR encephalitis between May 1, 2008 and January 2013 31. In addition, 25 patients with non inflammatory neurological diseases and 9 patients with Borrelia burgdorferi disease were included as controls. The concentration of CXCL13 was determined by enzyme-linked immunosorbent assay.

Compared with the controls, 70% of patients with early anti NMDAR encephalitis had elevated levels of CXCL13 in CSF (>7, pg/mL), but serum samples did not have this trend. High concentration of CXCL13 in cerebrospinal fluid is associated with fever or pain in the prodromal stage, limited response to treatment, clinical recurrence, and the synthesis of NMDAR antibody in the sheath. After the start of treatment evaluation of 2-6 months showed that 10 patients with limited treatment response (15), showed increased levels of CXCL13 in CSF; and patients who respond well to treatment (13 in total), were not elevated CXCL13 conditions.

6 of the 12 patients had elevated levels of CSF CXCL13 during the relapse period, of which 3 patients were at normal levels prior to CXCL13. In the brain, mononuclear cells infiltrating around the blood vessels, and microglia scattered in the parenchyma, can express CXCL13.

The results suggest that in 70% of patients with anti NMDAR encephalitis, the increase in CXCL13 concentration in CSF is associated with the synthesis of intrathecal NMDAR antibodies. A long-term or secondary increase in CXCL13 levels is associated with limited response to treatment and disease recurrence. In conclusion, CXCL13 is an effective biomarker and may be used clinically to guide the treatment and prognosis of NMDAR encephalitis.

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