In this article, Dr. Morten Blaabjerg of Odense University and Dr. Daniel Kondziella of Copenhagen University Hospital discuss chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). CLIPPERS is an inflammatory condition of unknown etiology, primarily involving the brainstem and cerebellum. Symptoms of CLIPPERS include subacute onset of brainstem symptoms–most prominently, ataxia, diplopia, dysarthria, and altered facial sensation. The core radiological features are punctate or curvilinear perivascular gadolinium enhancement, typically in the pons and adjacent cerebellum. Neuropathological examination of affected areas reveals a perivascular lymphocytic inflammation with predominant CD4+ T cells. Clinical and radiological features respond to high-dose corticosteroid treatment, but continuous oral corticosteroid treatment and steroid-sparing agents are needed to prevent relapses and to reduce the risk for subsequent parenchymal atrophy and chronic ataxia. The differential diagnosis for CLIPPERS is broad, and a careful workup is needed.
To view the complete article, click here and log in.