A Seronegative CCPD Misclassified as MS: A Practical Lesson in B-Cell-Driven Disease Stabilized With Rituximab

Abstract

Background

Combined central and peripheral demyelination (CCPD) is a rare immune-mediated disorder involving both central and peripheral myelin. Recent evidence suggests that CCPD may represent a distinct clinical and immunological entity rather than a coincidental overlap between multiple sclerosis (MS) and chronic inflammatory demyelinating polyneuropathy (CIDP).

Case Presentation

We report a 63-year-old seronegative female patient who had initially been followed with a diagnosis of MS and was later re-evaluated as having CCPD after developing new peripheral symptoms. Cerebrospinal fluid analysis revealed elevated protein levels with negative oligoclonal bands. Autoantibody screening, including anti-MOG, anti-MAG, AQP4, and NF155, was negative. MRI showed confluent periventricular and juxtacortical lesions consistent with central involvement. The patient showed clinical stability with no further radiological progression after rituximab therapy over an 8-month follow-up period.

Conclusion

This case supports the existence of a seronegative, B-cell-mediated variant of CCPD and highlights the potential role of rituximab in achieving disease stabilization. Recognition of CCPD as a distinct diagnostic entity may prevent misclassification as MS and guide appropriate immunotherapy strategies.