Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome: Clinical and Radiological Considerations

Fabio Pilato; Marisa Distefano; Rosalinda Calandrelli

doi:10.3389/fneur.2020.00034

Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome: Clinical and Radiological Considerations

Front Neurol. 2020 Feb 14:11:34. doi: 10.3389/fneur.2020.00034. eCollection 2020.

Authors

Fabio Pilato^{1

2}, Marisa Distefano³, Rosalinda Calandrelli^{1

4}

Affiliations

¹ Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.
² UOC Neurologia, Dipartimento di Scienze dell'invecchiamento, Neurologiche, Ortopediche e Della Testa-Collo, Rome, Italy.
³ UOC Neurologia e UTN, Ospedale Belcolle, Viterbo, Italy.
⁴ UOC Radiologia e Neuroradiologia, Dipartimento di Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, Rome, Italy.

Abstract

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.

Keywords: PRES; RCVS; call-fleming syndrome; magnetic resonance imaging; posterior reversible encephalopathy syndrome; reversible cerebral vasoconstriction syndrome; reversible posterior leukoencephalopathy syndrome.

Publication types

Review