BACKGROUND Childhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease in children. CASE PRESENTATION We present a case of a 17-year-old boy with recurrent ischemic events over a short time period. Diagnosis of angiography positive cPACNS was made based on neuroimaging findings while secondary causes or mimics of CNS vasculitis were meticulously excluded. The patient exhibited rapid deterioration of his condition with poor initial response to immunosuppressive treatment. CONCLUSIONS Recognition of cPACNS remains a challenge because of rarity of disease, unexplained etiopathogenesis, protean clinical presentation, as well as lack of specific laboratory and neuroimaging markers.

中文翻译：

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一名 17 岁男孩以缺血性中风为主要表现的中枢神经系统原发性血管炎的急进表现

背景儿童原发性中枢神经系统血管炎 (cPACNS) 是一种越来越多的儿童炎症性脑病。病例介绍 我们介绍了一个 17 岁男孩在短时间内反复发生缺血事件的病例。血管造影阳性 cPACNS 的诊断是基于神经影像学检查结果，同时仔细排除继发性原因或 CNS 血管炎的模拟。患者表现出病情迅速恶化，对免疫抑制治疗的初始反应不佳。结论 由于疾病罕见、病因不明、临床表现多变以及缺乏特定的实验室和神经影像学标志物，cPACNS 的识别仍然是一个挑战。