CLIPPERS syndrome

Abstract

CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a recently described rare disease affecting the central nervous system. It is characterized by subacute development of symptoms of lesions predominantly in the brain stem and cerebellum, by specific magnetic resonance imaging (MRI) changes, perivascular lymphocytic infiltration in the brain substance and a good response to glucocorticoid (GC) therapy. The paper describes CLIPPERS syndrome in a patient who has been followed up in a clinic for 10 years. During this period, different variants of clinical diagnosis have been considered. The final diagnosis was made only when comparing the clinical course and manifestations of the disease, MRI data, as well as the reaction to GC therapy and its discontinuation. Literature data and diagnostic criteria of this disease are presented.

Keywords

• clippers syndrome
• multiple sclerosis
• magnetic resonance imaging
• lymphocytic infiltration
• pons varolii
• lymphoma
• glioma
• vasculitides