Pilato Fabio, Distefano Marisa, Calandrelli Rosalinda
Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.
UOC Neurologia, Dipartimento di Scienze dell'invecchiamento, Neurologiche, Ortopediche e Della Testa-Collo, Rome, Italy.
Front Neurol. 2020 Feb 14;11:34. doi: 10.3389/fneur.2020.00034. eCollection 2020.
Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are relatively uncommon neurological disorders, but their detection has been increasing mainly due to clinical awareness and spreading of magnetic resonance imaging (MRI). Because these syndromes share some common clinical and radiologic features and occasionally occur in the same patient, misdiagnosis may occur. PRES is characterized by varied neurological symptoms including headache, impaired visual acuity or visual field deficit, confusion, disorders of consciousness, seizures, and motor deficits often associated to peculiar neuroradiological pattern even if uncommon localization and ischemic or hemorrhagic lesions were described. RCVS is a group of diseases typically associated with severe headaches and reversible segmental vasoconstriction of cerebral arteries, often complicated by ischemic or hemorrhagic stroke. Pathophysiological basis of PRES and RCVS are still debated but, because they share some risk factors and clinical features, a possible common origin has been supposed. Clinical course is usually self-limiting, but prognosis may fluctuate from complete recovery to death due to complications of ischemic stroke or intracranial hemorrhage. Neuroradiological techniques such as digital angiography and MRI are helpful in the diagnostic pathway and a possible prognostic role of MRI has been suggested. This review will serve to summarize clinical, neuroradiological features and controversies underlying both syndromes that may mislead the diagnostic pathway and their possible relationship with pathophysiology, clinical course, and prognosis.
后部可逆性脑病综合征(PRES)和可逆性脑血管收缩综合征(RCVS)是相对罕见的神经系统疾病,但由于临床意识的提高和磁共振成像(MRI)的普及,它们的检出率一直在上升。由于这些综合征有一些共同的临床和放射学特征,且偶尔会出现在同一患者身上,因此可能会发生误诊。PRES的特征是多种神经系统症状,包括头痛、视力障碍或视野缺损、意识模糊、意识障碍、癫痫发作和运动功能障碍,这些症状通常与特殊的神经放射学模式相关,尽管也有罕见部位以及缺血性或出血性病变的描述。RCVS是一组通常与严重头痛和脑动脉节段性可逆性血管收缩相关的疾病,常并发缺血性或出血性中风。PRES和RCVS的病理生理基础仍存在争议,但由于它们有一些共同的危险因素和临床特征,因此推测可能有共同的起源。临床病程通常是自限性的,但预后可能因缺血性中风或颅内出血的并发症而从完全恢复到死亡不等。数字血管造影和MRI等神经放射学技术在诊断过程中很有帮助,并且有人提出MRI可能具有预后作用。本综述将总结这两种综合征的临床、神经放射学特征以及可能误导诊断过程的争议点,以及它们与病理生理学、临床病程和预后的可能关系。