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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.

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/f7b5/10382238/20e7da12fe31/CRINM2023-5811243.001.jpg

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