背景后部可逆性脑病综合征(PRES)是一种缺乏特征和神秘的综合征。尽管一贯表现为神经系统血管源性水肿,这种疾病与多种触发因素、神经症状和自然史有关。病例报告报告一位25岁的非裔美国女性,表现为精神状态改变及双侧皮质性失明。神经影像发现在小脑、顶叶和枕叶有血管源性水肿。她的PRES伴有高血压急诊、肾功能衰竭和房间隔增生(培养阴性心内膜炎)。所有3种病因都得到了解决,病人开始恢复。在康复过程中,病人经历了大脑的视觉变形,将她的整个环境想象成卡通的形式。治疗2周后,患者恢复至基线健康状态,随访神经影像学显示血管源性水肿消失。结论:本病例在术前恢复期、脑变性视力以及一种新的潜在联系(培养阴性的房间隔心内膜炎)中呈现罕见的分类现象。该报告还强调了如何明确评估恢复前的患者(皮质盲)是否患有脑变性视和Charles Bonnet综合征——这可能会使患者感到痛苦。 Lastly, the atypical presentation of cerebellar vasogenic edema in our patient validates existing literature that PRES does not have a uniform picture and is not well served by its current name or proposed diagnostic criteria. Therefore, renaming the disorder to reversible vasogenic edema syndrome and derestricting the diagnostic criteria, may prevent clinicians from being discouraged when faced with diagnosing PRES in the face of atypical findings.

参考

PubMed