Brain Pathol. 2010 Jan;20(1):261-4. doi: 10.1111/j.1750-3639.2009.00346.x.
A 7 year old Chinese boy died of a rapidly progressive encephalopathy after influenza infection. MRI showed bilateral and symmetrical lesions including the thalamus and brainstem tegmentum. The pathology of necrosis and vasculopathy were in keeping with acute necrotizing encephalopathy (ANE). ANE was first described in Japan and carries a high mortality and morbidity. A vasculopathy with breakdown of the blood-brain-barrier was incriminated but the pathogenesis remained obscure and autopsy studies have been limited. A review of the literature showed only nine postmortem reports in the acute stage. Symmetrical brain necrosis always involved the thalamus followed by the tegmentum of the pons and other regions. Exudative vasculopathy was commonly observed and often accompanied by endothelial cell necrosis. In the present case there was inflammatory fibrinoid vasculitis which has not been previously described.
一名 7 岁中国男孩在流感感染后继发进行性脑病死亡。MRI 显示双侧、对称性丘脑和脑桥被盖病变。坏死和血管病变的病理学改变符合急性坏死性脑病(ANE)。ANE 最初在日本被描述,具有高死亡率和发病率。血管病变伴血脑屏障破坏被归咎于此,但发病机制仍不清楚,尸检研究也很有限。文献复习显示,急性期仅有 9 例尸检报告。对称性脑坏死总是累及丘脑,其次是脑桥被盖和其他区域。渗出性血管病变很常见,常伴有内皮细胞坏死。在本病例中,存在以前未描述的炎症纤维蛋白样血管炎。