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后部可逆性脑病综合征

Posterior reversible encephalopathy syndrome.

作者信息

Lamy C, Oppenheim C, Mas J L

机构信息

Department of Neurology, Université Paris Descartes, Hôpital Sainte-Anne, Paris, France.

Department of Neurology, Université Paris Descartes, Hôpital Sainte-Anne, Paris, France.

出版信息

Handb Clin Neurol. 2014;121:1687-701. doi: 10.1016/B978-0-7020-4088-7.00109-7.

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a recently proposed cliniconeuroradiologic entity with several well-known causes, such as hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressive drugs, as well as some causes more recently described. PRES is characterized by neuroimaging findings of reversible vasogenic subcortical edema without infarction. The pathogenesis is incompletely understood. Two opposing hypotheses are commonly cited, but the issue is controversial: (1) the current more popular theory suggests that severe hypertension exceeds the limits of autoregulation, leading to breakthrough brain edema; (2) the earlier original theory suggests that hypertension leads to cerebral autoregulatory vasoconstriction, ischemia, and subsequent brain edema. The clinical syndrome of PRES typically involves headache, encephalopathy, visual symptoms, and seizures. The clinical presentation is often nonspecific, and therefore the diagnosis of PRES has come to increasingly rely on magnetic resonance imaging (MRI) abnormalities consistent with PRES with documented recovery clinically and on repeated neuroimaging. The diagnosis has important therapeutic and prognostic implications because the reversibility of the clinical and radiologic abnormalities is contingent on the prompt control of blood pressure and/or discontinuing the offending drug.

摘要

后部可逆性脑病综合征(PRES)是一种最近提出的临床神经放射学实体,有多种已知病因,如高血压脑病、子痫、使用细胞毒性和免疫抑制药物,以及一些最近才描述的病因。PRES的特征是神经影像学表现为可逆性血管源性皮质下水肿且无梗死。其发病机制尚未完全明确。通常引用两种相反的假说,但这个问题存在争议:(1)目前更流行的理论认为,严重高血压超过了自动调节的限度,导致突破性脑水肿;(2)较早的原始理论认为,高血压导致脑自动调节性血管收缩、缺血及随后的脑水肿。PRES的临床综合征通常包括头痛、脑病、视觉症状和癫痫发作。临床表现往往不具特异性,因此PRES的诊断越来越依赖于与PRES相符的磁共振成像(MRI)异常,并有临床记录的恢复情况以及重复的神经影像学检查。该诊断具有重要的治疗和预后意义,因为临床和放射学异常的可逆性取决于血压的迅速控制和/或停用致病药物。

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