CLIPPER is a chronic inflammatory disorder in the CNS, which is characterized by MRI appearance of punctate and curvilinear gadolinium enhancement that involve the pons and the cerebellum and exquisite response to steroid. We report a patient presented with clinical and radiological features suggestive of CLIPPERS. However, despite the initial response to steroid, there were dramatic changes in the course of his disease that were conducive to considering another diagnosis. We searched PubMed using word (CLIPPERS) till December 2018. The pathogenesis, clinical manifestations, imaging features, treatment and prognosis of this disorder are summarized. A review of the literature for cases of CLIPPERS demonstrated a subset of patients who later discovered to have an alternative pathology. Indeed, clinicians should be scrupulous to diagnose this disease based solely on the clinical and radiological findings and they should have a lower threshold of having a brain biopsy.

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