Is there a role for B cells in chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids? A case report

Publication/Presentation Date

3-1-2019

Abstract

Background

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently defined inflammatory disorder of the central nervous system, prominently involving the brainstem. This condition is diagnosed based on clinical presentation, imaging findings and robust response to steroids, along with histopathology.

Case presentation

Here, we describe a case of a 47‐year‐old woman who presented with gait ataxia, bilateral leg weakness and urinary retention. Initial imaging studies were unremarkable, but on follow up she was found to have multiple enhancing lesions in the left cerebellar peduncle associated with a clinical relapse. Several weeks later, and after being treated with ocrelizumab, a B‐cell targeted therapy, she developed new neurological symptoms and was found to have new lesions on brain imaging. She was diagnosed with probable CLIPPERS. She was started on azathioprine and remained asymptomatic with normal brain imaging for several months.

Conclusions

Despite adequate depletion of circulating B cells, the patient continued to have relapses associated with CLIPPERS. This supports the notion that targeted peripheral B‐cell therapy does not stop the progression of CLIPPERS.

Volume

10

Issue

2

First Page

142

Last Page

144

Comments

Case Report

Disciplines

Neurology

Department(s)

Department of Medicine, Department of Medicine Faculty, Department of Medicine Fellows and Residents

Document Type

Article

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