• Lei Zhang, Dan Zhao, Jia Li, Jingqi Feng, Qinghui Zhang, Li Liu, Jingxin Liu, and Jiajun Chen.
• Department of Neurology, China-Japan Union Hospital of Jilin University, Changchun, Jilin.
• Medicine (Baltimore). 2021 Jun 4; 100 (22): e26090e26090.

RationaleChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a chronic inflammatory disorder of the central nervous system. It is characterized by the appearance on magnetic resonance imaging of punctate and curvilinear gadolinium enhancement in the pons and cerebellum, and is exquisitely responsive to steroid treatment. The etiology of CLIPPERS remains unclear, although its pathogenesis reflects immune-mediated processes. The accurate diagnosis of this disease is very important for both its management and prognosis.Patient ConcernsA 43-year-old woman presented with clinical and radiological features suggestive of CLIPPERS. Whole-exome sequencing of the patient's DNA revealed 76 mutations.DiagnosesThe patient was clinically diagnosed with CLIPPERS.InterventionsHormone therapy was administered intravenously upon hospitalization and then gradually reduced to an oral dose.OutcomesThe clinical symptoms and imaging manifestations of the patient improved rapidly. This patient was followed up for more than 1 year, and there has been no recurrence or aggravation.LessonsA gene variation library of CLIPPERS syndrome was established, which lays the foundation for the further accumulation of data, and will allow the etiology and pathogenesis of the disease to be explored.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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