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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综合征):一例报告及文献复习

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review.

作者信息

Shrestha Nripesh Man, Acharya Niranjan, Desar Rabindra

机构信息

Civil Service Hospital, Kathmandu, Nepal.

出版信息

Case Rep Neurol Med. 2023 Jul 21;2023:5811243. doi: 10.1155/2023/5811243. eCollection 2023.

DOI:10.1155/2023/5811243
PMID:37520771
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10382238/
Abstract

CLIPPERS is a rare, chronic inflammatory neurological syndrome affecting multiple regions of the brain including the brainstem, cerebellum, and spinal cord. More than 100 cases have been documented globally since its initial description in 2010. This article reports the first case of the CLIPPERS syndrome in Nepal. Clinical and radiological evidences of the patient lead to the diagnosis of this disease. Brain MRI reveals punctate and curvilinear gadolinium enhancement in the pons and cerebellum, which is diagnostic for the disease. Steroid therapy has been reported to be effective in treating CLIPPERS syndrome. Although its pathophysiology indicates an immune-mediated process, the etiology is yet unknown. The treatment and prognosis of this illness depend on an early and accurate diagnosis.

摘要

CLIPPERS是一种罕见的慢性炎症性神经综合征,可影响大脑的多个区域,包括脑干、小脑和脊髓。自2010年首次描述以来,全球已记录了100多例病例。本文报告了尼泊尔首例CLIPPERS综合征病例。患者的临床和影像学证据导致了该疾病的诊断。脑部MRI显示脑桥和小脑中点状和曲线状钆增强,这对该疾病具有诊断意义。据报道,类固醇疗法对治疗CLIPPERS综合征有效。尽管其病理生理学表明是一种免疫介导的过程,但其病因尚不清楚。这种疾病的治疗和预后取决于早期准确的诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7b5/10382238/80544f73bd37/CRINM2023-5811243.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7b5/10382238/20e7da12fe31/CRINM2023-5811243.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7b5/10382238/80544f73bd37/CRINM2023-5811243.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7b5/10382238/20e7da12fe31/CRINM2023-5811243.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f7b5/10382238/80544f73bd37/CRINM2023-5811243.002.jpg

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A case report of CLIPPERS syndrome and literature review.CLIPPERS 综合征病例报告及文献复习。
Medicine (Baltimore). 2021 Jun 4;100(22):e26090. doi: 10.1097/MD.0000000000026090.
2
Unusual CLIPPERS presentation and role of MRI examination in the proper diagnostic assessment: A case report.不典型的中枢神经系统特发性炎症性脱髓鞘假瘤(CLIPPERS)表现及MRI检查在正确诊断评估中的作用:一例报告
Eur J Radiol Open. 2019 Jun 6;6:212-214. doi: 10.1016/j.ejro.2019.05.005. eCollection 2019.
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Effective treatment of CLIPPERS with long-term use of rituximab.
长期使用利妥昔单抗对伴有皮质下梗死和白质脑病的常染色体隐性遗传性脑动脉病(CLIPPERS)进行有效治疗。
Neurol Neuroimmunol Neuroinflamm. 2018 Mar 1;5(3):e448. doi: 10.1212/NXI.0000000000000448. eCollection 2018 May.
4
Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).类固醇反应性脑桥淋巴细胞血管周围浸润伴炎症的诊断标准(CLIPPERS)。
Brain. 2017 Sep 1;140(9):2415-2425. doi: 10.1093/brain/awx200.
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Therapeutic Approaches in CLIPPERS.慢性淋巴细胞性炎症伴脑桥血管周围强化症(CLIPPERS)的治疗方法
Curr Treat Options Neurol. 2017 May;19(5):17. doi: 10.1007/s11940-017-0455-4.
6
Chronic hepatitis B infection presenting with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): a case report.慢性乙型肝炎感染伴桥脑周围血管强化的慢性淋巴细胞性炎症且对类固醇有反应(CLIPPERS):一例报告
J Med Case Rep. 2015 Nov 19;9:266. doi: 10.1186/s13256-015-0750-1.
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Effective antituberculous therapy in a patient with CLIPPERS: New insights into CLIPPERS pathogenesis.对伴有慢性淋巴细胞性炎症伴脑桥血管周围强化症(CLIPPERS)患者的有效抗结核治疗:对CLIPPERS发病机制的新见解
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