CLIPPERS syndrome: A case report in a Chinese patient with juxtacortical lesions

Abstract Background : Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory central nervous system (CNS) disease with unknown pathogenesis and polymorphic clinical and radiological manifestations. However, juxtacortical lesions have scarcely reported in patients with CLIPPERS. Case presentation In this study, we report 32 year old man with complaint of intermittent dizziness, disordered fluency and walking difficulties for 2 weeks. The neuroimaging revealed multiple punctuate hyperintensities on T2-weighted images, with pepper-like gadolinium enhancement in the typical lesions of brainstem, cerebellum, periventricular as well as untypical lesions of juxtacortical area. After other similar diseases were carefully ruled out, the patient was diagnosed as CLIPPERS and exhibited a good response to immunosuppressive therapy on the acute phase and after 6 months of follow up. Conclusions : The results reported here broaden the known radiologic features for this rare disorder. A long-term follow-up to rule out other potential differential diseases is essential.