Presence of identical B-cell clone in both cerebrospinal fluid and tumor
tissue in a patient with Opsoclonus-myoclonus syndrome associated with
neuroblastoma
Abstract
Opsoclonus-myoclonus syndrome associated with neuroblastoma (OMS-NB) is
a refractory paraneoplastic syndrome which often remain neurological
sequelae, and detailed pathogenesis has remained elusive. We encountered
a pediatric patient with OMS-NB treated by immunosuppressed therapy who
showed anti-glutamate receptor δ2 antibody and increased B-cells in
cerebrospinal fluid (CSF), and multiple lymphoid follicles containing
abundant B-cells in tumor tissue. Unbiased B-cell receptor repertoire
analysis revealed identical B-cell clone was identified as the dominant
clone in both CSF and tumor tissue. These identical B-cell clone may
contribute to the pathogenesis of OMS-NB. Our results could facilitate
the establishment of pathogenesis-based treatment strategies for OMS-NB.