Montelongo Janet A, Ellis Carley A, Cheng Jennifer J, Fields Timothy A, Fels Elliott Daffolyn Rachael, Qureshi Abid Y
Department of Neurology, University of Kansas Medical Center, Kansas City, KS, USA.
Department of Neurosurgery, University of Kansas Medical Center, Kansas City, KS, USA.
Neurohospitalist. 2025 May 12:19418744251336501. doi: 10.1177/19418744251336501.
A 51-year-old woman presented with acute onset of a severe headache, and was found to have diffuse subarachnoid hemorrhage with prominent cisternal and left cortical convexity blood on head computed tomography. The first 2 conventional angiograms were negative for aneurysm, but a third angiogram revealed a mycotic aneurysm of a distal left middle cerebral artery branch. Brain biopsy, associated with clipping of the aneurysm, demonstrated pathology consistent with vasculitis. Over the course of a month, she developed diffuse, serpiginous dolichoectasia of the cerebral arteries. Further investigation into the cause of vasculitis supported a diagnosis of either eosinophilic granulomatosis with polyangiitis (EGPA) or IgG4-Related Disease (IgG4-RD). The following clinical pathologic conference discusses the diagnostic challenges in discriminating between these 2 diseases, particularly in the setting of secondary angiitis of the central nervous system.
一名51岁女性因急性剧烈头痛就诊，头颅计算机断层扫描显示为弥漫性蛛网膜下腔出血，脑池和左侧皮质凸面有明显出血。最初的2次传统血管造影未发现动脉瘤，但第3次血管造影显示左大脑中动脉远端分支有一个霉菌性动脉瘤。脑活检结合动脉瘤夹闭术，病理结果符合血管炎。在一个月的病程中，她出现了脑动脉弥漫性、匐行性迂曲扩张。对血管炎病因的进一步调查支持嗜酸性肉芽肿性多血管炎（EGPA）或IgG4相关性疾病（IgG4-RD）的诊断。以下临床病理讨论会讨论了鉴别这两种疾病的诊断挑战，特别是在中枢神经系统继发性血管炎的情况下。
