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类固醇反应性桥脑周围血管增强的慢性淋巴细胞性炎症(CLIPPERS):一例报告并文献复习

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): A case report and review of literature.

作者信息

Reddy Soma Madhan, Lath Rahul, Swain Meenakshi, Ranjan Alok

机构信息

Department of Neurosurgery, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh, India.

Department of Pathology, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh, India.

出版信息

Ann Indian Acad Neurol. 2015 Jul-Sep;18(3):345-7. doi: 10.4103/0972-2327.152085.

DOI:10.4103/0972-2327.152085
PMID:26425017
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4564474/
Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of central nervous system with distinct clinical and radiological features. The etiopathogenesis of this rare entity remains to be understood. The histopathological findings closely resemble chronic inflammatory diseases like sarcoidosis and malignancies like lymphoma. With advancements in serology, immunopathology and radiology CLIPPERS is identified as a distinct entity that differs considerably in its clinical presentation, immunopathology, radiological findings and response to steroids. We describe a case that presented to us with progressive quadriparesis and lower cranial nerve deficits whose radiological and pathological findings were consistent with CLIPPERS. The patient had a good outcome with long term immunosuppression.

摘要

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,具有独特的临床和放射学特征。这种罕见疾病的病因发病机制仍有待明确。其组织病理学表现与结节病等慢性炎症性疾病以及淋巴瘤等恶性肿瘤相似。随着血清学、免疫病理学和放射学的发展,CLIPPERS被确定为一种独特的疾病实体,其临床表现、免疫病理学、放射学表现及对类固醇的反应均有很大差异。我们描述了一例以进行性四肢瘫和低位颅神经功能缺损就诊的病例,其放射学和病理学表现与CLIPPERS相符。该患者通过长期免疫抑制治疗取得了良好疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/085ff4a54e03/AIAN-18-345-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/787570cfa044/AIAN-18-345-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/9c7522d05575/AIAN-18-345-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/085ff4a54e03/AIAN-18-345-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/787570cfa044/AIAN-18-345-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/9c7522d05575/AIAN-18-345-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b67/4564474/085ff4a54e03/AIAN-18-345-g003.jpg

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本文引用的文献

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Need for prolonged immunosupressive therapy in CLIPPERS--a case report.CLIPPERS 患者需要长期免疫抑制治疗——1 例报告。
BMC Neurol. 2013 May 24;13:49. doi: 10.1186/1471-2377-13-49.
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Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients.12例连续性慢性淋巴细胞性炎症伴脑桥血管周围强化症(CLIPPERS,对类固醇治疗有效的疾病)患者的长期预后。
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Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
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A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with initial normal magnetic resonance imaging.一例初始磁共振成像正常、对类固醇有反应的伴有脑桥血管周围强化的慢性淋巴细胞性炎症新病例。
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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).伴有桥脑血管周围强化的慢性淋巴细胞性炎症对类固醇反应良好(CLIPPERS)。
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