暴发性原发性中枢神经系统血管炎的不典型表现。
Atypical presentation of fulminant primary central nervous system angiitis.
机构信息
Department of Critical Care Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada.
Department of Pathology & Laboratory Medicine, University of Calgary, Calgary, AB T2N 1N4, Canada.
出版信息
J Neuroimmunol. 2019 May 15;330:1-4. doi: 10.1016/j.jneuroim.2019.01.019. Epub 2019 Feb 1.
BACKGROUND
Primary Angiitis of the Central Nervous System (PACNS) is a rare cause of CNS vasculitis that should be included as part complete differential diagnosis, especially in cases with suggestive imaging findings and an absence of secondary causes for CNS vasculitis.
CASE PRESENTATION
We describe a case of a 47-year-old previously healthy Caucasian male presenting with rapid progression of encephalopathy and fevers. Extensive infectious, autoimmune, and imaging workups were unrevealing. A diagnosis of PACNS was made posthumously on histopathology.
CONCLUSIONS
PACNS is a challenging diagnosis owing to frequent discrepancies between radiologic and histopathologic findings. Tissue biopsy is key to diagnosing PACNS.
背景
原发性中枢神经系统血管炎（PACNS）是中枢神经系统血管炎的罕见病因，应作为完整鉴别诊断的一部分，特别是在有提示性影像学发现且无中枢神经系统血管炎继发原因的情况下。
病例介绍
我们描述了一例 47 岁的既往健康的白人男性，表现为进行性脑病和发热。广泛的感染性、自身免疫性和影像学检查均未见异常。死后组织病理学诊断为 PACNS。
结论
PACNS 的诊断具有挑战性，因为影像学和组织病理学发现经常存在差异。组织活检是诊断 PACNS 的关键。
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