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以头痛为唯一症状的类固醇反应性桥脑周围血管强化慢性淋巴细胞性炎症(CLIPPERS):一例报告及文献复习

Headache As the Sole Symptom in Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS): A Case Report and Literature Review.

作者信息

Abdelkader Omar, Abuzenah Hamza, Shuhaiber Hans

机构信息

Department of Neurology, Mayo Clinic, Jacksonville, USA.

Internal Medicine, Yarmouk University, Amman, JOR.

出版信息

Cureus. 2024 Jul 11;16(7):e64310. doi: 10.7759/cureus.64310. eCollection 2024 Jul.

DOI:10.7759/cureus.64310
PMID:39130962
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11316347/
Abstract

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare central nervous system inflammatory condition usually presenting with a range of symptoms, including ataxia, diplopia, dysarthria, seizures, and headaches. We present a unique case of a 22-year-old woman exhibiting headache as the sole symptom. Imaging and biopsy confirmed the diagnosis, and initial steroid treatment provided relief, though it relapsed on tapering. Long-term management with low-dose steroids and mycophenolate mofetil achieved remission. This case highlights the importance of recognizing atypical presentations of CLIPPERS, emphasizing the need for prompt diagnosis and appropriate treatment plans to improve patient outcomes. Further research is necessary to enhance our understanding and management of CLIPPERS.

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)是一种罕见的中枢神经系统炎性疾病,通常表现出一系列症状,包括共济失调、复视、构音障碍、癫痫发作和头痛。我们报告了一例独特的病例,一名22岁女性仅以头痛为唯一症状。影像学检查和活检确诊了该病,初始类固醇治疗缓解了症状,但减量时病情复发。低剂量类固醇和霉酚酸酯的长期治疗实现了病情缓解。该病例突出了认识CLIPPERS非典型表现的重要性,强调了及时诊断和制定适当治疗方案以改善患者预后的必要性。需要进一步研究以加深我们对CLIPPERS的理解和管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/de6bf6dc2519/cureus-0016-00000064310-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/0f559c00bb18/cureus-0016-00000064310-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/2447e9abacab/cureus-0016-00000064310-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/de6bf6dc2519/cureus-0016-00000064310-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/0f559c00bb18/cureus-0016-00000064310-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/2447e9abacab/cureus-0016-00000064310-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d89f/11316347/de6bf6dc2519/cureus-0016-00000064310-i03.jpg

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本文引用的文献

1
The pathogenesis hypothesis and research progress of CLIPPERS: A literature review.CLIPPERS 的发病机制假说和研究进展:文献综述。
Medicine (Baltimore). 2023 Mar 17;102(11):e33211. doi: 10.1097/MD.0000000000033211.
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Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies.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)的临床特征、治疗及预后:对100项研究中140例患者的全面系统评价
Mult Scler Relat Disord. 2022 Dec;68:104112. doi: 10.1016/j.msard.2022.104112. Epub 2022 Aug 18.
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MOG autoimmunity mimicking CLIPPERS syndrome: Case report and literature review.
模仿CLIPPERS综合征的MOG自身免疫性疾病:病例报告及文献综述
J Neuroimmunol. 2022 Jun 15;367:577875. doi: 10.1016/j.jneuroim.2022.577875. Epub 2022 Apr 22.
4
Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) With Extrapontine and Pulmonary Involvement: Case Report of a Rare Presentation and Review of Literature.类固醇反应性脑桥边缘带淋巴细胞血管周炎伴慢性淋巴细胞炎症(CLIPPERS)伴脑外和肺部受累:一种罕见表现的病例报告及文献复习。
Neurol India. 2022 Jan-Feb;70(1):405-408. doi: 10.4103/0028-3886.338665.
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Brain biopsy in patients with CLIPPERS syndrome: why and when.伴有慢性淋巴细胞性炎症伴脑桥血管周围强化症(CLIPPERS)综合征患者的脑活检:原因及时机
Ther Adv Neurol Disord. 2022 Jan 30;15:17562864211062821. doi: 10.1177/17562864211062821. eCollection 2022.
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CLIPPERS, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A challenge in neurological practice, clinical landmarks (Review).CLIPPERS,即对类固醇有反应的桥脑周围血管增强的慢性淋巴细胞性炎症:神经科临床实践中的一项挑战,临床标志(综述)
Exp Ther Med. 2021 Oct;22(4):1191. doi: 10.3892/etm.2021.10625. Epub 2021 Aug 17.
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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.
8
CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS.CLIPPERS 及其模拟物:新的 CLIPPERS 诊断标准的评估。
J Neurol Neurosurg Psychiatry. 2019 Sep;90(9):1027-1038. doi: 10.1136/jnnp-2018-318957. Epub 2019 May 9.
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Headache. 2018 Mar;58(3):458-459. doi: 10.1111/head.13232. Epub 2017 Nov 28.
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Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids, with Cranial and Caudal Extension.伴有脑桥血管周围强化的慢性淋巴细胞性炎症,对类固醇治疗有效,病变有头端和尾端延伸
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