Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 11, 2015
Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS)
- Pathogenesis: unknown
- Clinical Presentation: Dysarthria, ataxia and diplopia. Usually responds to treatment with steroids.
- Pathology: Perivascular spaces infiltrated with lymphocytes
- Key Diagnostic Features:
- Numerous patchy FLAIR signal hyperintensities, typically at the pontocerebellar junction
- Minimal enhancement
- Can sometimes extend cephalad to involve the midbrain, basal ganglia, and the periventricular white matter.
- Occasionally involves the spinal cord.
- No restricted diffusion
- Imaging findings reversible upon treatment with steroids
- Diagnosis on imaging is largely one of exclusion.
- DDx:
- Lymphoma
- Histiocytosis
- Encephalitis
- Vasculitis
- Sarcoidosis
- Rx: Corticosteroids, if no response to immunosuppressive therapy
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