Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
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DOI：
10.1093/brain/awq164
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发表时间：
2010-09-01
期刊：
影响因子：
14.5
通讯作者：
Keegan, B. Mark
中科院分区：
文献类型：
--
作者：
Pittock, Sean J.;Debruyne, Jan;Keegan, B. Mark
许多中枢神经系统炎症性疾病的分类和病理机制尚不清楚。在这篇文章中，我们报告了8例临床和放射学上不同的桥脑优势型脑脊髓炎患者，我们将其命名为‘慢性淋巴细胞性炎症伴对类固醇反应的脑桥血管周围强化’(Clippers)。从1999年到2009年，在美国梅奥诊所和比利时根特大学医院对患者进行了临床、放射和病理评估。从临床开始的中位随访期为22个月(范围7-144个月)。患者接受了广泛的实验室(血清和脑脊液)、放射和病理检查(结膜、经支气管镜和脑活检)，以寻找炎性中枢神经系统疾病的原因。所有8名患者(5名女性，3名男性)都表现为发作性复视或面部感觉异常，随后出现脑干症状，偶尔出现脊髓病症状，并对大剂量糖皮质激素有良好的初始反应。所有患者均有对称性曲线强化，遍及脑桥和延髓、桥臂、小脑、中脑，偶尔还可见脊髓。放射学改善伴随着糖皮质激素的临床反应。患者通常在糖皮质激素减量后病情恶化，并需要长期使用糖皮质激素或其他免疫抑制治疗。4例患者活检组织的神经病理显示血管周围白质，主要是T淋巴细胞，无肉芽肿、感染、淋巴瘤或血管炎。脑桥血管周围对类固醇的反应增强的慢性淋巴细胞性炎症是一种明确的慢性炎症性中枢神经系统疾病，可接受免疫抑制治疗。受影响的中枢神经系统病变以T细胞为主的炎症病理，临床和放射学对免疫抑制治疗的反应与免疫介导的过程是一致的。
The classification and pathological mechanisms of many central nervous system inflammatory diseases remain uncertain. In this article we report eight patients with a clinically and radiologically distinct pontine-predominant encephalomyelitis we have named 'chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids' (CLIPPERS). The patients were assessed clinically, radiologically and pathologically at Mayo Clinic, USA and Ghent University Hospital, Belgium from 1999 to 2009. Median follow-up duration from clinical onset was 22 months (range 7-144 months). Patients underwent extensive laboratory (serum and cerebrospinal fluid), radiological and pathological testing (conjunctival, transbronchial and brain biopsies) to search for causes of an inflammatory central nervous system disorder. All eight patients (five female, three male) presented with episodic diplopia or facial paresthesias with subsequent brainstem and occasionally myelopathic symptoms and had a favourable initial response to high dose glucocorticosteroids. All patients had symmetric curvilinear gadolinium enhancement peppering the pons and extending variably into the medulla, brachium pontis, cerebellum, midbrain and occasionally spinal cord. Radiological improvement accompanied clinical response to glucocorticosteroids. Patients routinely worsened following glucocorticosteroid taper and required chronic glucocorticosteroid or other immunosuppressive therapy. Neuropathology of biopsy material from four patients demonstrated white matter perivascular, predominantly T lymphocytic, infiltrate without granulomas, infection, lymphoma or vasculitis. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a definable, chronic inflammatory central nervous system disorder amenable to immunosuppressive treatment. The T cell predominant inflammatory pathology in affected central nervous system lesions and the clinical and radiological response to immunosuppressive therapies is consistent with an immune-mediated process.