Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with intracranial Epstein-Barr virus infection: A Case Report.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with intracranial Epstein-Barr virus infection: A Case Report.
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DOI：
10.1097/md.0000000000005377
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发表时间：
2016-11
期刊：
影响因子：
1.6
通讯作者：
Jiang W
中科院分区：
文献类型：
--
作者：
Ma Y;Sun X;Li W;Li Y;Kang T;Yang X;Jiang W
慢性淋巴细胞性炎症伴对类固醇敏感的脑桥血管周围增强（CLIPPERS）是一种中枢神经系统（CNS）炎症性疾病，具有独特的临床、放射学和病理学特征。CLIPPERS的病理生理机制尚不清楚，相关报道较少。尽管据报道放射性病变主要位于脑桥、脑桥臂和小脑，但最近有报道称其他邻近结构（例如白色物质和脊髓）也是CLIPPERS的受累区域。在这项研究中，我们报告一个CLIPPERS的情况下，提出颅内EB病毒（EBV）感染和弥漫性白色物质的参与。一位37岁男性在26岁时被诊断为纵隔霍奇金淋巴瘤（淋巴细胞优势型），经治疗后获得完全缓解，11年未复发。患者入院时有7个月的精神障碍病史，20天的步态和肢体共济失调、吞咽困难和咳嗽病史。CLIPPERS的诊断是基于磁共振成像（MRI）中双侧脑桥、基底节、中脑、脑桥臂和弥漫性白色物质的点状和结节状增强病变，以及双侧顶叶脑活检显示的血管周围和实质区域的CD 3 + T淋巴细胞炎性浸润。此外，我们的病人表现出良好的反应，类固醇治疗，并保持免费复发5个月。重要的是，我们发现颅内EB病毒感染的病人。CLIPPERS可能是一种自身免疫性疾病，颅内EBV感染增加了EBV相关自身免疫与CLIPPERS发病机制相关的可能性。
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder in the central nervous system (CNS) with distinct clinical, radiological, and pathological features. The pathophysiology of CLIPPERS still remains unclear and the reports are quite few. Although the radiological lesions were reported to be located predominantly in the pons, brachium pontis, and cerebellum, other adjacent structures such as the white matter and spinal cord were very recently reported as involved regions in CLIPPERS. In this study, we report a case of CLIPPERS presenting with intracranial Epstein–Barr virus (EBV) infection and diffuse white matter involvement. A 37-year-old male was diagnosed with mediastinal Hodgkin's lymphoma (lymphocyte predominance type) at the age of 26, and then obtained complete remission after treatment and remained free of relapse for 11 years. He was admitted with 7 months’ history of mental disorder, and 20 days’ history of gait and limb ataxia, dysphagia, and cough. The diagnosis of CLIPPERS was established based on the findings of punctate and nodular enhancing lesions in the bilateral pons, the basal ganglia, the mid-brain, the pontine brachium, and diffuse white matter in magnetic resonance imaging (MRI), together with CD3+ T-lymphocytic inflammatory infiltration in perivascular and parenchymal area revealed by bilateral parietal lobe brain biopsy. Also, our patient exhibited a good response to steroid therapy and remained free of relapse for 5 months. Importantly, we found intracranial Epstein–Barr virus infection in this patient. CLIPPERS might be an autoimmune disorder, and intracranial EBV-infection raises the possibility that EBV-associated autoimmunity is associated with CLIPPERS pathogenesis.