What is the management of posterior reversible encephalopathy syndrome (PRES)?

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

The management of PRES requires immediate cessation of the triggering agent, stringent blood pressure control, and symptomatic treatment of neurological manifestations, with most cases showing complete resolution within 2 weeks with appropriate supportive care. 1

Clinical Presentation and Diagnosis

• PRES presents with acute neurological deficits including altered consciousness, visual disturbances (including blindness), headaches, and seizures 1
• Diagnosis is confirmed by T2-weighted MRI showing hyperintensities in bilateral parietal-occipital lobes, predominantly affecting white matter 1
• The pathophysiology involves disruption of the blood-brain barrier due to endothelial injury from abrupt blood pressure changes, leading to vasogenic edema 1

Management Algorithm

Step 1: Identify and Address Underlying Causes

• Discontinue the offending agent immediately (e.g., anticancer therapy, immunosuppressants like cyclosporine) 1
• Common triggers include:
  • Pre-existing arterial hypertension 1
  • Renal impairment 1
  • Autoimmune diseases 1
  • High-dose antineoplastic therapy 1
  • Allogenic stem-cell transplantation 1
  • Solid organ transplantation 1
  • Immunosuppression (particularly cyclosporine) 1

Step 2: Blood Pressure Management

• Implement very stringent blood pressure control as the cornerstone of management 1
• Target gradual reduction of blood pressure to avoid cerebral hypoperfusion 1
• Monitor blood pressure frequently during the acute phase 1

Step 3: Seizure Management

• Administer antiepileptic treatment for patients who develop seizures 1
• Benzodiazepines may be used for symptomatic treatment of acute seizures 1
• Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits 1

Step 4: Electrolyte Management

• Correct electrolyte imbalances if present 1
• Monitor renal function, especially in patients with pre-existing renal impairment 1

Step 5: Supportive Care

• Provide appropriate ICU-level care for patients with severe symptoms 2
• Monitor neurological status closely for signs of deterioration 3

Step 6: Follow-up and Monitoring

• Repeat neuroimaging to confirm resolution of vasogenic edema 4
• Most cases resolve within 2 weeks with appropriate supportive management 1, 5
• Decision to reintroduce prior anticancer therapy should be made on an individual basis after complete resolution 1

Special Considerations

• For PRES associated with immunotherapy, follow the Society for Immunotherapy of Cancer guidelines for immune-related adverse events 1
• In cases with persistent symptoms, consider alternative diagnoses or complications 1
• For PRES during ECMO, neurological consultation is recommended for acute neurological changes 1

Prognosis

• With prompt recognition and management, PRES is typically reversible with a favorable prognosis 3, 5
• Complete spontaneous remission occurs in most cases without sequelae 1
• Early detection and management are key factors for rapid recovery and good outcomes 5
• In rare cases, neurological sequelae including long-term epilepsy may persist 3

Common Pitfalls to Avoid

• Delaying treatment while awaiting confirmatory imaging - initiate management based on clinical suspicion 4
• Overly aggressive blood pressure reduction, which may lead to cerebral hypoperfusion 3
• Failure to identify and discontinue the triggering agent 1
• Missing concomitant conditions that may complicate management (e.g., sepsis, metabolic disturbances) 1
• Overlooking the need for close neurological monitoring during the acute phase 5