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The Peppered Brainstem 

The Peppered Brainstem
Chapter:
The Peppered Brainstem
Author(s):

Aaron E. Miller

, Tracy M. DeAngelis

, Michelle Fabian

, and Ilana Katz Sand

DOI:
10.1093/med/9780190693190.003.0026
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date: 29 January 2020

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory syndrome of the CNS. Patients present with subacute, progressive symptoms that usually localize to the brainstem. MRI shows a characteristic appearance of diffuse involvement that is greatest in the pons and surrounding areas and is associated with a “peppered” pattern of gadolinium enhancement. Pathologically, CLIPPERS is characterized by the perivascular and parenchymal infiltration of CD4+ T cells as well as by B cells and activated microglia. Patients with CLIPPERS generally respond to steroid treatment but are at high risk for rebound when steroids are discontinued. Long-term immunosuppression is often required in order to avoid relapse. The prognosis for CLIPPERS is variable, though many good outcomes have been reported.

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