Journal article

Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome


Authors listFuehlhuber, V.; Bick, S.; Kirsten, A.; Hahn, A.; Gerriets, I.; Tschernatsch, M.; Kaps, M.; Preissner, K. T.; Blaes, F.; Altenkaemper, S.

Publication year2009

Pages87-91

JournalJournal of Neuroimmunology

Volume number210

Issue number1-2

ISSN0165-5728

eISSN1872-8421

DOI Linkhttps://doi.org/10.1016/j.jneuroim.2009.03.006

PublisherElsevier


Abstract

Childhood opsoclonus-myoclonus syndrome (OMS) occurs idiopathic or, in association with a neuroblastoma, as a paraneoplastic syndrome. Since autoantibodies were identified in some patients. an autoimmune pathogenesis has been suspected. While the newly discovered B-cell activating factors BAFF and APRIL are involved in systemic autoimmune diseases, their association with neuroimmunological diseases is hardly understood. We here investigated the BAFF and APRIL levels in serum and cerebrospinal fluid (CSF) of OMS patients and their correlation with surface-binding autoantibodies.

BAFF and APRIL were both determined by ELISA. and autoantibodies to cerebellar granular neurons (CGN) have been investigated by flow cytometry in 17 OMS patients, 16 neuroblastoma (NB) patients, 13 controls and 11 children with inflammatory neurological diseases (IND).

BAFF, but no APRIL, was elevated in the CSF of OMS children and IND children. However, in contrast to IND patients, OMS patients did not have a blood-brain-barrier disturbance, indicating that BAFF was produced intrathecally in OMS patients, but not in IND patients. CSF BAFF levels showed a correlation with CSF CGN autoantibodies (r(2) = 0.58, p < 0.05).

These data indicate that an activated B-cell system in the cerebrospinal fluid is involved in the pathogenesis of OMS, and BAFF may be a candidate parameter for the activation of B-cell immune system. (C) 2009 Elsevier B.V. All rights reserved.




Citation Styles

Harvard Citation styleFuehlhuber, V., Bick, S., Kirsten, A., Hahn, A., Gerriets, I., Tschernatsch, M., et al. (2009) Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome, Journal of Neuroimmunology, 210(1-2), pp. 87-91. https://doi.org/10.1016/j.jneuroim.2009.03.006

APA Citation styleFuehlhuber, V., Bick, S., Kirsten, A., Hahn, A., Gerriets, I., Tschernatsch, M., Kaps, M., Preissner, K., Blaes, F., & Altenkaemper, S. (2009). Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome. Journal of Neuroimmunology. 210(1-2), 87-91. https://doi.org/10.1016/j.jneuroim.2009.03.006



Keywords


ATAXIAAUTOIMMUNITYB-cell activating factorOpsoclonus-myoclonus-syndromeParaneoplastic neurological syndromePARANEOPLASTIC OPSOCLONUSSJOGRENS-SYNDROMESYSTEMIC-LUPUS-ERYTHEMATOSUS

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