The paper presents a case of a 61-year-old patient with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), along with a detailed description of the diagnostic and therapeutic process, as well as radiological records reflecting the evolution of lesions depending on the steroid therapy used. We also refer to the latest literature reports on the aetiology, pathogenesis, diagnosis and treatment of this clinical entity, including its potential relationship with lymphoproliferative and lymphohistiocytic processes. Furthermore, we present a spectrum of diseases that can clinically and radiologically mimic CLIPPERS (CLIPPERS-mimics), along with the proposed differential diagnosis. Finally, the paper presents the recommended treatment strategy for both acute disease and a maintenance therapy, with particular emphasis on the role of steroid therapy.

CLIPPERS, autoimmune encephalitis, CLIPPERS-mimics, lymphoma, ataxia, cerebellar syndrome