title: Posterior reversible encephalopathy syndrome--Insight into pathogenesis, clinical variants and treatment approaches.
abstract: Posterior reversible encephalopathy syndrome is a rare clinicoradiological entity characterized by typical MRI findings located in the occipital and parietal lobes, caused by subcortical vasogenic edema. It was first described as a distinctive syndrome by Hinchey in 1996. Etiopathogenesis is not clear, although it is known that it is an endotheliopathy of the posterior cerebral vasculature leading to failed cerebral autoregulation, posterior edema and encephalopathy. A possible pathological activation of the immune system has been recently hypothesized in its pathogenesis. At clinical onset, the most common manifestations are seizures, headache and visual changes. Besides, tinnitus and acute vertigo have been frequently reported. Symptoms can be reversible but cerebral hemorrhage or ischemia may occur. Diagnosis is based on magnetic resonance imaging, in the presence of acute development of clinical neurologic symptoms and signs and arterial hypertension and/or toxic associated conditions with possible endotheliotoxic effects. Mainstay on the treatment is removal of the underlying cause. Further investigation and developments in endothelial cell function and in neuroimaging of cerebral blood flow are needed and will help to increase our understanding of pathophysiology, possibly suggesting novel therapies.
authors.affiliation: Department of Clinical Immunology, Sapienza University of Rome, Viale dell'Università, 37, 00161 Rome, Italy. Electronic address: guido.granata@alice.it.
authors.forename: Guido
authors.initials: G
authors.lastname: Granata
authors.affiliation: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico, 151, 00161 Rome, Italy. Electronic address: antonio.greco@uniroma1.it.
authors.forename: Antonio
authors.initials: A
authors.lastname: Greco
authors.affiliation: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico, 151, 00161 Rome, Italy. Electronic address: giannicolaiannella@hotmail.it.
authors.forename: Giannicola
authors.initials: G
authors.lastname: Iannella
authors.affiliation: Department of Clinical Immunology, Sapienza University of Rome, Viale dell'Università, 37, 00161 Rome, Italy. Electronic address: massimo.granata@uniroma1.it.
authors.forename: Massimo
authors.initials: M
authors.lastname: Granata
authors.affiliation: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico, 151, 00161 Rome, Italy. Electronic address: alessandramanno@hotmail.it.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Manno
authors.affiliation: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico, 151, 00161 Rome, Italy. Electronic address: ersilia.savastano@gmail.it.
authors.forename: Ersilia
authors.initials: E
authors.lastname: Savastano
authors.affiliation: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico, 151, 00161 Rome, Italy. Electronic address: giuseppemagliuloorl@yahoo.com.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Magliulo
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D007759:Labyrinth Diseases; D008279:Magnetic Resonance Imaging; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D011379:Prognosis
keywords: Acute vertigo; Cerebral edema; Hypertensive encephalopathy; Immune dysregulation; Posterior reversible leucoencephalopathy syndrome; Seizures; Tinnitus