Effective treatment of CLIPPERS with long-term use of rituximab

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare CNS inflammatory syndrome first described in 2010 by Pittock et al. Taieb et al. reported about 60 known cases throughout the world. The underlying pathogenesis of CLIPPERS has not been fully elucidated, and a neural autoantibody target has not been discovered. Steroid treatment is effective in treating the inflammatory component of the disease, but long-term treatment of CLIPPERS with steroids is limited by side effects. Steroid withdrawal is associated with recrudescence of the disease, and each relapse is associated with additional disability. Moderately efficacious steroid-sparing treatments include methotrexate, hydroxychloroquine, and cyclophosphamide. However, the long-term efficacy and safety of these agents in CLIPPERS remain to be determined. Anti-CD20 molecules benefit several antibody-mediated CNS inflammatory diseases. Herein, we describe a case of CLIPPERS successfully treated with rituximab (mouse chimeric anti-CD20 monoclonal antibody) over the course of 4 years. We describe imaging and histological features of this disease, as they are relevant to disease pathology and treatment (figure).