PDF(Pubmed)
Abstract：
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystem disease characterized by plasma cell dyscrasia and overproduction of vascular endothelial growth factor, which is related to disease activity. Recent treatment strategies have improved survival of patients suffering from this disorder; however, ischemic stroke remains a poor prognostic factor. POEMS patients with ischemic stroke frequently develop cerebral large artery stenosis/occlusion, followed by progressive stroke. Post literature review, we present an ischemic stroke case of quasi-moyamoya disease linked with this syndrome that was successfully treated with surgical revascularization. A 41-year-old woman diagnosed with POEMS syndrome developed progressive ischemic stroke due to quasi-moyamoya disease, despite decreased vascular endothelial growth factor level with lenalidomide and dexamethasone treatment. She underwent superficial temporal artery to middle cerebral artery bypass with encephalo-duro-myo-synangiosis bilaterally. The postoperative course was uneventful. Two years and five months after the stroke, neuroimaging demonstrated bypass patency, neovascularization after encephalo-duro-myo-synangiosis, and no recurrence of stroke. Our case is the first to report successful surgical revascularization for a POEMS patient. Surgical revascularization may be a useful treatment option for patients with quasi-moyamoya disease associated with POEMS syndrome, especially for those who develop refractory ischemic stroke despite reduced vascular endothelial growth factor level.
摘要：
POEMS（多发性神经病，器官肿大,内分泌病,单克隆丙种球蛋白病，和皮肤变化）综合征是一种罕见的多系统疾病，其特征是浆细胞异常和血管内皮生长因子的过度产生，这与疾病活动有关。最近的治疗策略改善了患有这种疾病的患者的生存率；然而，缺血性卒中仍是不良预后因素.POEMS缺血性卒中患者常发生脑大动脉狭窄/闭塞,其次是进展性中风。后文献综述，我们介绍了一例与该综合征相关的拟烟雾病的缺血性卒中病例，该病例通过手术血运重建成功治疗。一名41岁被诊断患有POEMS综合征的女性因准烟雾病而发展为进行性缺血性中风，尽管来那度胺和地塞米松治疗降低了血管内皮生长因子水平。她接受了颞浅动脉至大脑中动脉旁路术，并双侧伴有脑-硬-肌-血管增生。术后病程顺利。中风后两年零五个月，神经影像学显示搭桥通畅，脑-硬-肌-血管综合征后新生血管形成，而且没有中风复发.我们的病例是第一个报告POEMS患者成功进行手术血运重建的病例。对于与POEMS综合征相关的准烟雾病患者，手术血运重建可能是一种有用的治疗选择。特别是对于那些尽管血管内皮生长因子水平降低但仍发展为难治性缺血性卒中的患者。
