Department of Neurology, Patras University Hospital, Patras, Greece.
Department of Rheumatology, Patras University Hospital, Patras, Greece.
J Neuroimmunol. 2020 Apr 15;341:577190. doi: 10.1016/j.jneuroim.2020.577190. Epub 2020 Feb 13.
Childhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease in children.
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.
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.
儿童原发性中枢神经系统血管炎（cPACNS）是一种在儿童中日益被认识到的炎症性脑部疾病。
我们报告了一例 17 岁男孩在短时间内反复发作缺血性事件的病例。基于神经影像学发现，排除了中枢神经系统血管炎的继发性原因或类似疾病后，诊断为血管造影阳性 cPACNS。该患者病情迅速恶化，初始免疫抑制治疗反应不佳。
由于疾病罕见、病因不明、临床表现多样以及缺乏特异性实验室和神经影像学标志物，因此对 cPACNS 的认识仍然具有挑战性。
