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局灶性中枢神经系统血管炎表现为新发局灶性意识障碍性癫痫发作。

Focal CNS vasculitis masquerading as new-onset focal aware seizures.

机构信息

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

出版信息

BMJ Case Rep. 2023 Sep 27;16(9):e255535. doi: 10.1136/bcr-2023-255535.

DOI:10.1136/bcr-2023-255535
PMID:37758658
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10537825/
Abstract

Primary angiitis of the central nervous system (PACNS) is an autoimmune disease with myriad presentations in the form of headache, focal neurological deficits, seizures and rapid cognitive decline. Predilection or isolated and recurrent involvement of one hemisphere is rarely described in literature. This leads to low clinical suspicion and often delayed diagnosis of PACNS, if clinical and radiological involvement is focal. We describe the case of a young man presenting with new-onset focal seizures with imaging suggestive of focal involvement and the brain biopsy clinched an early diagnosis. The 1-year follow-up showed clinical and radiological resolution.

摘要

原发性中枢神经系统血管炎(PACNS)是一种自身免疫性疾病,有多种表现形式,如头痛、局灶性神经功能缺损、癫痫发作和快速认知能力下降。在文献中很少有描述单侧或孤立性、复发性受累的情况。这导致 PACNS 的临床怀疑度低,且如果临床和影像学表现为局灶性,往往会延迟诊断。我们描述了一位年轻男性的病例,他表现为新发局灶性癫痫发作,影像学提示局灶性受累,而脑活检则明确了早期诊断。1 年的随访显示临床和影像学均得到缓解。

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本文引用的文献

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Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.原发性中枢神经系统血管炎:系统评价和荟萃分析。
Neurol Neuroimmunol Neuroinflamm. 2021 Oct 18;8(6). doi: 10.1212/NXI.0000000000001093. Print 2021 Nov.
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Unilateral Relapsing Primary Angiitis of the CNS: An Entity Suggesting Differences in the Immune Response Between the Cerebral Hemispheres.单侧复发型中枢神经系统原发性血管炎:提示大脑半球间免疫反应存在差异的一种疾病实体。
Neurol Neuroimmunol Neuroinflamm. 2021 Jan 5;8(2). doi: 10.1212/NXI.0000000000000936. Print 2021 Mar 4.
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Primary CNS lymphoma initially diagnosed as vasculitis.最初被诊断为血管炎的原发性中枢神经系统淋巴瘤。
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Unihemispheric central nervous system vasculitis.单侧半球中枢神经系统血管炎。
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