Posterior Reversible Encephalopathy Syndrome

Curr Pain Headache Rep. 2021 Feb 25;25(3):19. doi: 10.1007/s11916-020-00932-1.

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. 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.

Keywords: Headache; Hypertensive encephalopathy; Immunosuppressive drugs; Reversible posterior encephalopathy syndrome; Vasogenic edema.

Publication types

  • Review

MeSH terms

  • Acute Chest Syndrome / epidemiology
  • Aminolevulinic Acid / analogs & derivatives
  • Anemia, Sickle Cell / epidemiology
  • Autoimmune Diseases / epidemiology
  • Blood-Brain Barrier / metabolism
  • Brain Edema / diagnostic imaging
  • Brain Edema / physiopathology
  • COVID-19 / epidemiology
  • Cerebrovascular Circulation / physiology
  • Cytokines / metabolism
  • Eclampsia / epidemiology
  • Endothelium / physiopathology*
  • Female
  • Headache / physiopathology*
  • Homeostasis / physiology
  • Humans
  • Hypertension / physiopathology
  • Magnetic Resonance Imaging
  • Posterior Leukoencephalopathy Syndrome / diagnostic imaging
  • Posterior Leukoencephalopathy Syndrome / epidemiology
  • Posterior Leukoencephalopathy Syndrome / physiopathology*
  • Posterior Leukoencephalopathy Syndrome / therapy
  • Pre-Eclampsia / epidemiology
  • Pregnancy
  • Prognosis
  • Renal Insufficiency / epidemiology
  • SARS-CoV-2
  • Seizures / physiopathology*
  • Vasospasm, Intracranial / physiopathology
  • Vision Disorders / physiopathology*

Substances

  • Cytokines
  • 2-amino-5-hydroxy-4-oxopentanoic acid
  • Aminolevulinic Acid