[Posterior reversible encephalopathy syndrome]

Med Klin Intensivmed Notfmed. 2016 Jun;111(5):417-24. doi: 10.1007/s00063-016-0175-7. Epub 2016 Jun 6.
[Article in German]

Abstract

Posterior reversible encephalopathy syndrome refers to a neurological disorder characterized by headache, disorders of consciousness, visual disturbances, epileptic seizures, and subcortical vasogenic edema. About two thirds of patients develop neurological symptoms, which are associated with blood pressure fluctuations. One hypothesis is that hypertensive episodes cause autoregulatory failure, and values above the upper limit of cerebral autoregulation result in a breakthrough followed by hyperperfusion and blood-brain barrier dysfunction. In another hypothesis, endothelial dysfunction triggered by numerous factors including preeclampsia, immunosuppressive agents, chemotherapeutics, sepsis, or autoimmune disorders is thought to be the key pathomechanism. Endo- or exogenic toxic agents including pharmacological substances, cytokines, or bacterial toxins are supposed to trigger endothelial activation and dysfunction resulting in the release of vasoconstrictors, pro-inflammatory mediators, and vascular leakage. Diagnosis is usually based on clinical and neuroimaging findings that frequently show a bilateral, symmetric, and parietooccipital pattern. However, the diagnosis can often only be confirmed during the course of disease after excluding important differential diagnoses. Currently, there is no specific treatment available. Lowering of arterial blood pressure and eliminating the underlying cause usually leads to an improvement of clinical and neuroradiological findings. Admission to a critical care unit is required in about 40 % of patients due to complicating conditions including status epilepticus, cerebral vasoconstriction, ischemia, or intracerebral hemorrhage. Prognosis is favorable; in the majority of patients neurological deficits and imaging findings resolve completely.

Keywords: Autoimmune diseases; Chemotherapy; Hypertensive encephalopathy; Imaging; Pre-eclampsia.

Publication types

  • Review

MeSH terms

  • Blood Pressure / physiology
  • Brain / blood supply
  • Capillary Leak Syndrome / diagnosis
  • Capillary Leak Syndrome / etiology
  • Capillary Leak Syndrome / physiopathology
  • Critical Care
  • Diagnosis, Differential
  • Endothelium, Vascular / physiopathology
  • Humans
  • Inflammation Mediators / blood
  • Posterior Leukoencephalopathy Syndrome / diagnosis*
  • Posterior Leukoencephalopathy Syndrome / etiology*
  • Posterior Leukoencephalopathy Syndrome / physiopathology
  • Posterior Leukoencephalopathy Syndrome / therapy
  • Vasoconstriction / physiology

Substances

  • Inflammation Mediators