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可逆性后部白质脑病综合征 (posterior reversible leukoencephalopathy syndrome, RPLS)是一种双侧大脑半球对称性白质可逆性水肿为主要表现的疾病。RPLS的概念于1996年由Hinchey首次提出。该病多见于恶性高血压或妊娠子痫、严重肾脏疾病、恶性肿瘤化疗以及各种器官组织移植后接受免疫抑制治疗的患者。临床主要表现为急性或亚急性起病, 症状包括头痛、精神行为异常、癫痫、皮质盲或其他视觉改变、小脑性共济失调等。RPLS典型的神经影像学改变是大脑后部皮质下为主的、双侧对称性、斑片状的血管源性水肿。头CT和MRI是RPLS常用的检查手段,病灶在CT上呈低密度,在T1 WI上呈等或低信号,在T2 WI上呈高信号,在FLAIR像上呈高信号。该病预后较好, 绝大多数病人神经系统症状能够完全恢复。
Almost two decades have elapsed since posterior reversible encephalopathy syndrome (PRES) was described in an influential case series. This usually reversible clinical syndrome is becoming increasingly recognised, in large part because of improved and more readily available brain imaging. Although the pathophysiological changes underlying PRES are not fully understood, endothelial dysfunction is a key factor. A diagnosis of PRES should be considered in the setting of acute neurological symptoms in patients with renal failure, blood pressure fluctuations, use of cytotoxic drugs, autoimmune disorders, or eclampsia. Characteristic radiographic findings include bilateral regions of subcortical vasogenic oedema that resolve within days or weeks. The presence of haemorrhage, restricted diffusion, contrast enhancement, and vasoconstriction are all compatible with a diagnosis. In most cases, PRES resolves spontaneously and patients show both clinical and radiological improvements. The range of symptoms that can comprise the syndrome might be broader than usually thought. In its mild form, this disorder might cause only one clinical symptom (headache or seizure) and radiographically might show few areas of vasogenic oedema or even normal brain imaging in some rare cases. In severe forms, PRES might cause substantial morbidity and even mortality, most often as a result of acute haemorrhage or massive posterior fossa oedema causing obstructive hydrocephalus or brainstem compression.
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来自： tu8tu > 《（PRES）可逆性后部脑病综合征》
