Lane Chris, Phadke Rahul, Howard Robin
Department of Neurology, Guys and St Thomas' Hospital, London, UK.
Department of Neuropathology, UCL Institute of Neurology, London, UK.
BMJ Case Rep. 2014 Jun 25;2014:bcr2014204117. doi: 10.1136/bcr-2014-204117.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described central nervous system inflammatory condition. In this case report we describe a patient initially with features consistent with this syndrome, who represented with seizures (not previously reported in this syndrome) and corresponding prominent cortical involvement on imaging (also not previously noted). Owing to diagnostic uncertainty, cerebral biopsy was performed revealing histology consistent with CLIPPERS, excluding other differentials. Following a further brainstem relapse, this patient was treated with high-dose steroids, subsequently switched to a tapering oral regime and now, azathioprine, a steroid-sparing agent. She remains well on this.
类固醇反应性桥脑周围血管强化的慢性淋巴细胞性炎症（CLIPPERS）是一种最近描述的中枢神经系统炎性疾病。在本病例报告中，我们描述了一名最初具有与该综合征相符特征的患者，该患者以癫痫发作（此综合征中先前未报告过）为表现，且影像学显示相应的显著皮质受累（先前也未被注意到）。由于诊断存在不确定性，进行了脑活检，结果显示组织学与CLIPPERS相符，排除了其他鉴别诊断。在脑干再次复发后，该患者接受了大剂量类固醇治疗，随后改为逐渐减量的口服方案，现在使用硫唑嘌呤，一种类固醇替代药物。她目前病情稳定。
