CLIPPERS Syndrome: A Rare Inflammatory Disease of the Central Nervous System
CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a rare, chronically inflammatory disease of the central nervous system (CNS) that primarily affects the brainstem—especially the pons—and the cerebellum. The condition was first described in 2010 by Pittock et al., and has since been diagnosed in only a small number of patients worldwide (Pittock et al., 2010). What is CLIPPERS? The acronym describes the core features of the disease: Chronic : The course of the disease is long-lasting or relapsing. Lymphocytic : The inflammation is characterized by infiltration of lymphocytes. Pontine Perivascular Enhancement : MRI shows punctate or small patchy contrast enhancement around blood vessels in the pons. Steroid-Responsive : Symptoms and MRI changes improve significantly under corticosteroid treatment. Symptoms The clinical presentation is variable and depends on the location of the i...