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 Bcells 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.

Keywords:

• Identical B-cell clone
• neuroblastoma
• Opsoclonus–myoclonus syndrome
• unbiased B-cell receptor repertoire analysis

Authors’ contributions

K.N. wrote the manuscript. M.T., Y.S., T.F., R.K., K.N., S.S., M.F. and R.A. provided patient care. H.I. and S.N. provided pathological analysis. Y.T. provided anti-glutamate receptor δ2 analysis. Y.I. was the principal investigator and takes primary responsibility for the paper. T.W. was the chief of the department and provided advice on this study.

Disclosure statement

No potential conflict of interest was reported by the authors.

Funding

The author(s) reported there is no funding associated with the work featured in this article.