Abstract
To study aberrant B cell trafficking into the CSF in opsoclonus-myoclonus syndrome (OMS), chemoattractants CXCL13 and CXCL12, and B cell frequency and CXCR5 expression, were evaluated. CSF CXCL13 concentration and the CSF/serum ratio were higher in untreated OMS than controls, related directly to OMS severity and inversely to OMS duration, and correlated with CSF B cell frequency and oligoclonal bands. CXCL12 showed the opposite pattern. Selective accumulation of CXCR5+ memory B cells in CSF was found. In ACTH-treated OMS, CXCL13, but not CXCL12, was lower. These data implicate the chemokine/chemoreceptor pair CXCL13/CXR5 in B cell recruitment to the CNS in OMS. CXCL13 and CXCL12 may serve as reciprocal biomarkers of disease activity, but CXCL13 also had utility as a treatment biomarker.
Trial registration:
ClinicalTrials.gov NCT00806182.
Copyright © 2011 Elsevier B.V. All rights reserved.
Publication types
-
Research Support, Non-U.S. Gov't
MeSH terms
-
Adolescent
-
Adrenal Cortex Hormones / therapeutic use
-
Adrenocorticotropic Hormone / pharmacology
-
Adrenocorticotropic Hormone / therapeutic use
-
Analysis of Variance
-
Antigens, CD / metabolism
-
B-Lymphocytes / drug effects
-
B-Lymphocytes / metabolism*
-
Case-Control Studies
-
Cell Movement / drug effects
-
Chemokine CXCL12 / cerebrospinal fluid
-
Chemokine CXCL12 / metabolism
-
Chemokine CXCL13 / cerebrospinal fluid
-
Chemokine CXCL13 / metabolism*
-
Child
-
Child, Preschool
-
Cross-Sectional Studies
-
Enzyme-Linked Immunosorbent Assay
-
Female
-
Flow Cytometry
-
Humans
-
Immunoglobulins, Intravenous / therapeutic use
-
Infant
-
Longitudinal Studies
-
Male
-
Opsoclonus-Myoclonus Syndrome / cerebrospinal fluid*
-
Opsoclonus-Myoclonus Syndrome / drug therapy
-
Receptors, CXCR5 / metabolism*
Substances
-
Adrenal Cortex Hormones
-
Antigens, CD
-
CXCL12 protein, human
-
CXCL13 protein, human
-
CXCR5 protein, human
-
Chemokine CXCL12
-
Chemokine CXCL13
-
Immunoglobulins, Intravenous
-
Receptors, CXCR5
-
Adrenocorticotropic Hormone
Associated data
-
ClinicalTrials.gov/NCT00806182