CLIPPERS: A case report with radiology, three serial biopsies and a literature review

Abstract

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare central nervous system inflammatory disorder primarily affecting the brainstem and cerebellum. We report a case of CLIPPERS in a 45-year-old man presenting with left facial numbness and dizziness. Imaging studies were conducted repeatedly over an 8-year follow up period. Given diagnostic uncertainty in the early stages of the disease, three serial biopsies were obtained, which together with the clinical and radiological findings led to the diagnosis. This case highlights the diagnostic challenges regarding the rare entity of CLIPPERS and discusses the main differential diagnoses that are necessary to consider. Additionally, some of the atypical features of this case, including the presenting finding of a large, solidly enhancing lesion on radiological imaging and prominent plasma cells on pathology, contribute expanding the spectrum of appearances for CLIPPERS.

Good response to rituximab in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids presenting as tumor‐like infratentorial lesion

“…2 Tumor-like lesions in CLIPPERS represent an extremely rare finding that can be observed both at the onset or during the course of the disease. [3][4][5] Given the significant clinical impact of such tumefactive variants, the use of anti-CD20 monoclonal antibodies, which have entered powerfully in the therapeutic scenario of several central nervous system (CNS) inflammatory diseases, might be considered a suitable option in selected cases.…”