Clinic of Neurology, Jessenius Faculty of Medicine in Martin, Comenius University Bratislava, Slovak Republic.
Biomedical Center Martin, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Slovak Republic.
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2023 Sep;167(3):303-308. doi: 10.5507/bp.2022.013. Epub 2022 Mar 21.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory central nervous system (CNS) disorder, chiefly involving the brainstem, especially the pons. The diagnosis is challenging, requires careful exclusion of alternative diagnoses and a targeted therapeutic approach. CLIPPERS is known to respond well to corticosteroids, but the treatment needs to be long-term and can cause significant side-effects. Moreover, subsequent corticosteroid withdrawal often leads to a relapse. It has been suggested that anti-CD20 molecules could benefit several antibody-mediated CNS inflammatory diseases, including CLIPPERS.
This paper describes two cases of CLIPPERS. The first demonstrates the benefit of early introduction of corticosteroids with side effects in cases of long-term use. The second demonstrates the efficacy of ocrelizumab (anti-CD20 molecule) in a severe course of CLIPPERS.
These two cases bring attention to this rare, often misdiagnosed but treatable disease.
伴有桥脑血管周围强化的慢性淋巴细胞性炎症反应伴类固醇反应性（CLIPPERS）是一种罕见的中枢神经系统（CNS）炎症性疾病，主要累及脑干，特别是脑桥。该诊断具有挑战性，需要仔细排除其他诊断，并采取针对性的治疗方法。CLIPPERS 已知对皮质类固醇反应良好，但治疗需要长期进行，并可能引起明显的副作用。此外，随后的皮质类固醇停药常导致复发。有人认为抗 CD20 分子可能有益于包括 CLIPPERS 在内的几种抗体介导的中枢神经系统炎症性疾病。
本文描述了两例 CLIPPERS 病例。第一个病例表明，在长期使用皮质类固醇的情况下，早期引入皮质类固醇并出现副作用可带来益处。第二个病例表明奥瑞珠单抗（抗 CD20 分子）在 CLIPPERS 的严重病程中有效。
这两个病例引起了人们对这种罕见的、经常被误诊但可治疗的疾病的关注。
