Posterior Reversible Encephalopathy Syndrome

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Abstract

Purpose of Review

This review provides an updated discussion on the clinical presentation, diagnosis and radiographic features, mechanisms, associations and epidemiology, treatment, and prognosis of posterior reversible encephalopathy syndrome (PRES). Headache is common in PRES, though headache associated with PRES was not identified as a separate entity in the 2018 International Classification of Headache Disorders. Here, we review the relevant literature and suggest criteria for consideration of its inclusion.

Recent Findings

COVID-19 has been identified as a potential risk factor for PRES, with a prevalence of 1–4% in patients with SARS-CoV-2 infection undergoing neuroimaging, thus making a discussion of its identification and treatment particularly timely given the ongoing global pandemic at the time of this writing.

Summary

PRES is a neuro-clinical syndrome with specific imaging findings. The clinical manifestations of PRES include headache, seizures, encephalopathy, visual disturbances, and focal neurologic deficits. Associations with PRES include renal failure, preeclampsia and eclampsia, autoimmune conditions, and immunosuppression. PRES is theorized to be a syndrome of disordered autoregulation and endothelial dysfunction resulting in preferential hyperperfusion of the posterior circulation. Treatment typically focuses on treating the underlying cause and removal of the offending agents.

Related collections

Most cited references 66

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Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition

(2018)

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A reversible posterior leukoencephalopathy syndrome.

J Hinchey, C. Chaves, B Appignani … (1996)

In some patients who are hospitalized for acute illness, we have noted a reversible syndrome of headache, altered mental functioning, seizures, and loss of vision associated with findings indicating predominantly posterior leukoencephalopathy on imaging studies. To elucidate this syndrome, we searched the log books listing computed tomographic (CT) and magnetic resonance imaging (MRI) studies performed at the New England Medical Center in Boston and Hôpital Sainte Anne in Paris; we found 15 such patients who were evaluated from 1988 through 1994. Of the 15 patients, 7 were receiving immunosuppressive therapy after transplantation or as treatment for aplastic anemia, 1 was receiving interferon for melanoma, 3 had eclampsia, and 4 had acute hypertensive encephalopathy associated with renal disease (2 with lupus nephritis, 1 with acute glomerulonephritis, and 1 with acetaminophen-induced hepatorenal failure). Altogether, 12 patients had abrupt increases in blood pressure, and 8 had some impairment of renal function. The clinical findings included headaches, vomiting, confusion, seizures, cortical blindness and other visual abnormalities, and motor signs. CT and MRI studies showed extensive bilateral white-matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres, but the changes often involved other cerebral areas, the brain stem, or the cerebellum. The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn or the dose was reduced. In all 15 patients, the neurologic deficits resolved within two weeks. Reversible, predominantly posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed. The findings on neuroimaging are characteristic of subcortical edema without infarction.

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Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions.

Jennifer E Fugate, Alejandro A Rabinstein (2015)

Almost two decades have elapsed since posterior reversible encephalopathy syndrome (PRES) was described in an influential case series. This usually reversible clinical syndrome is becoming increasingly recognised, in large part because of improved and more readily available brain imaging. Although the pathophysiological changes underlying PRES are not fully understood, endothelial dysfunction is a key factor. A diagnosis of PRES should be considered in the setting of acute neurological symptoms in patients with renal failure, blood pressure fluctuations, use of cytotoxic drugs, autoimmune disorders, or eclampsia. Characteristic radiographic findings include bilateral regions of subcortical vasogenic oedema that resolve within days or weeks. The presence of haemorrhage, restricted diffusion, contrast enhancement, and vasoconstriction are all compatible with a diagnosis. In most cases, PRES resolves spontaneously and patients show both clinical and radiological improvements. The range of symptoms that can comprise the syndrome might be broader than usually thought. In its mild form, this disorder might cause only one clinical symptom (headache or seizure) and radiographically might show few areas of vasogenic oedema or even normal brain imaging in some rare cases. In severe forms, PRES might cause substantial morbidity and even mortality, most often as a result of acute haemorrhage or massive posterior fossa oedema causing obstructive hydrocephalus or brainstem compression.

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Author and article information

Contributors

Alexandra N. Gewirtz:

ORCID: http://orcid.org/0000-0002-1807-9226

ang9221@nyp.org

Journal

Journal ID (nlm-ta): Curr Pain Headache Rep

Journal ID (iso-abbrev): Curr Pain Headache Rep

Title: Current Pain and Headache Reports

Publisher: Springer US (New York )

ISSN (Print): 1531-3433

ISSN (Electronic): 1534-3081

Publication date (Electronic): 25 February 2021

Publication date (Print): 2021

Volume: 25

Issue: 3

Electronic Location Identifier: 19

Affiliations

Department of Neurology, New York-Presbyterian Hospital, Weill Cornell Medicine, 525 East 68th Street, New York, NY 10065 USA

Author information

Alexandra N. Gewirtz http://orcid.org/0000-0002-1807-9226

Article

Publisher ID: 932

DOI: 10.1007/s11916-020-00932-1

PMC ID: 7905767

PubMed ID: 33630183

SO-VID: 2e9bc90e-afee-4341-9148-0246fd996896

License:

This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

History

Date accepted : 29 December 2020

Custom metadata

ScienceOpen disciplines: Anesthesiology & Pain management

Keywords: reversible posterior encephalopathy syndrome,immunosuppressive drugs,vasogenic edema,hypertensive encephalopathy,headache

Data availability:

ScienceOpen disciplines: Anesthesiology & Pain management

Keywords: reversible posterior encephalopathy syndrome, immunosuppressive drugs, vasogenic edema, hypertensive encephalopathy, headache

Posterior Reversible Encephalopathy Syndrome

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