Posterior Reversible Encephalopathy Syndrome: Clinicoradiological Spectrum and Therapeutic Strategies

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome of encephalopathy, headache, visual disturbance, and seizures. In most cases, symptoms present acutely or subacutely in the setting of accelerated hypertension, eclampsia, autoimmune disease, immunosuppressive treatment, or cancer chemotherapy. One essential feature of PRES is the presence of reversible cerebral vasogenic edema that has a predominantly posterior distribution on brain imaging. Atypical imaging features are commonly described, including involvement of the anterior brain or brainstem and the coexistence of ischemia or hemorrhage. In most cases, both clinical and radiological findings are reversible, although permanent imaging abnormalities and residual neurological sequelae can be seen in a minority of patients. The syndrome is thought to be caused by a breakdown of the blood-brain barrier and an extravasation of the intravascular fluid. Treatment of hypertension and seizures, and withdrawal of causative agents are the mainstays of therapy in PRES.

Clinical Management of Posterior Reversible Encephalopathy Syndrome after Allogeneic Hematopoietic Stem Cell Transplantation: A Case Series and Review of the Literature

“…Therapy for PRES typically includes control of the blood pressure, treatment of seizures, and withdrawal or reduction of the offending drugs [9,11,14,33]. In the present series, clinical symptoms dissolved within 2-5 days after prompt correction of the blood pressure and discontinuation of CNI, except in 1 patient who died due to septic multiorgan failure.…”

“…Therapy usually includes control of the blood pressure, treatment of seizures, and withdrawal of the causative agents [9,11,14,33]. However, discontinuation of immunosuppressive therapy may reverse the neurotoxicity but implies a risk of graft rejection or development of GvHD.…”

Reversible Confluent Deep White Matter Abnormalities: A New Variant of Posterior Reversible Encephalopathy Syndrome

“…A diagnosis of PRES is based on the following essential findings: (1) the presence of significant hypertension or other typical triggering factors such as cancer treatment or toxemia of pregnancy; (2) the occurrence of at least 1 of the following neurological symptoms: headache, seizure, visual disturbance, and mental status change; (3) the image findings of reversible predominantly subcortical vasogenic edema [2]. In the current report, both of our patients presented with malignant hypertension.…”

“…Such a classical pattern is typically seen in approximately 70% of patients [2]. In recent years, a variety of atypical imaging patterns of PRES have been recognized and reported, including asymmetrical, unilateral, and isolated lobar involvement pattern [3].…”

Akutes Atemnotsyndrom und posteriores reversibles Enzephalopathie-Syndrom nach Behandlung mit Rituximab

“…Die Ätiologie ist nicht vollständig geklärt, es wird jedoch diskutiert, dass der Verlust der zerebralen Autoregulation ein wichtiger Schritt sein könnte, der dann entweder zu lokaler Hypertonie und Durchbruchblutungen führt oder zu anomaler reflektorischer Vasokonstriktion mit anschließender lokaler Ischämie und vasogener Ödembildung [11,12,13]. PRES ist mit einer Reihe von Krankheitsbildern in Zusammenhang gebracht worden, darunter Eklampsie, maligne Hypertonie, systemischer Lupus erythematosus und maligne Erkrankungen, potenziell außerdem mit verschiedenen Arzneimitteln, insbesondere Calcineurin-Inhibitoren, Cyclophosphamid und verschiedenen monoklonalen Wirkstoffe [14,15,16].…”