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Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome.
- Source :
-
Journal of neuroimmunology [J Neuroimmunol] 2009 May 29; Vol. 210 (1-2), pp. 87-91. Date of Electronic Publication: 2009 Mar 31. - Publication Year :
- 2009
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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.
- Subjects :
- Antibody Formation immunology
Autoantibodies analysis
B-Cell Activating Factor blood
B-Cell Activating Factor cerebrospinal fluid
Biomarkers analysis
Biomarkers blood
Biomarkers cerebrospinal fluid
Blood-Brain Barrier immunology
Cerebellar Diseases cerebrospinal fluid
Cerebellar Diseases physiopathology
Cerebellum immunology
Cerebellum pathology
Cerebellum physiopathology
Child, Preschool
Female
Humans
Male
Opsoclonus-Myoclonus Syndrome blood
Opsoclonus-Myoclonus Syndrome cerebrospinal fluid
Predictive Value of Tests
Subarachnoid Space immunology
Subarachnoid Space metabolism
Tumor Necrosis Factor Ligand Superfamily Member 13 analysis
Tumor Necrosis Factor Ligand Superfamily Member 13 blood
Up-Regulation immunology
Autoantibodies cerebrospinal fluid
B-Cell Activating Factor analysis
Cerebellar Diseases immunology
Lymphocyte Activation immunology
Opsoclonus-Myoclonus Syndrome immunology
Subjects
Details
- Language :
- English
- ISSN :
- 1872-8421
- Volume :
- 210
- Issue :
- 1-2
- Database :
- MEDLINE
- Journal :
- Journal of neuroimmunology
- Publication Type :
- Academic Journal
- Accession number :
- 19339060
- Full Text :
- https://doi.org/10.1016/j.jneuroim.2009.03.006