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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1-2
pubmed:dateCreated
2009-5-18
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
1872-8421
pubmed:author
pubmed:issnType
Electronic
pubmed:day
29
pubmed:volume
210
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
87-91
pubmed:meshHeading
pubmed-meshheading:19339060-Antibody Formation, pubmed-meshheading:19339060-Autoantibodies, pubmed-meshheading:19339060-B-Cell Activating Factor, pubmed-meshheading:19339060-Biological Markers, pubmed-meshheading:19339060-Blood-Brain Barrier, pubmed-meshheading:19339060-Cerebellar Diseases, pubmed-meshheading:19339060-Cerebellum, pubmed-meshheading:19339060-Child, Preschool, pubmed-meshheading:19339060-Female, pubmed-meshheading:19339060-Humans, pubmed-meshheading:19339060-Lymphocyte Activation, pubmed-meshheading:19339060-Male, pubmed-meshheading:19339060-Opsoclonus-Myoclonus Syndrome, pubmed-meshheading:19339060-Predictive Value of Tests, pubmed-meshheading:19339060-Subarachnoid Space, pubmed-meshheading:19339060-Tumor Necrosis Factor Ligand Superfamily Member 13, pubmed-meshheading:19339060-Up-Regulation
pubmed:year
2009
pubmed:articleTitle
Elevated B-cell activating factor BAFF, but not APRIL, correlates with CSF cerebellar autoantibodies in pediatric opsoclonus-myoclonus syndrome.
pubmed:affiliation
Dept. of Neurology, Justus-Liebig-University, Giessen, Germany.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't