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

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

The cornerstone of PRES management is immediate identification and removal of the triggering factor, combined with stringent blood pressure control to prevent further brain injury and promote recovery. 1

Step 1: Identify and Address Underlying Causes

• Immediately discontinue any potential offending agents such as anticancer therapies, immunosuppressants (particularly cyclosporine), or medications like infliximab 1
• Common triggers include pre-existing hypertension, renal impairment, autoimmune diseases, high-dose antineoplastic therapy, stem-cell transplantation, solid organ transplantation, and immunosuppression 1
• Obtain MRI (gold standard) showing characteristic hyperintensities in bilateral parietal-occipital lobes, predominantly affecting white matter, to confirm diagnosis 1

Step 2: Blood Pressure Management

• Implement very stringent blood pressure control with gradual reduction to avoid cerebral hypoperfusion 1
• Position patient with head elevated at 30 degrees to help reduce intracranial pressure 2
• Monitor blood pressure frequently during the acute phase to ensure it remains within a safe range 1
• Target blood pressure range should consider that optimal range to prevent cerebrovascular damage is 135-150 mmHg systolic and 70-79 mmHg diastolic 3

Step 3: Seizure Management

• Administer antiepileptic treatment for patients who develop seizures 1
• Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits 1
• For acute seizures, benzodiazepines may be used with careful monitoring 1
• In cases of severe PRES with uncontrolled seizures, consider ICU admission for closer monitoring 4

Step 4: Supportive Care

• For patients with declining level of consciousness, obtain urgent ICU assessment for airway protection and ventilatory support 2
• Implement aspiration precautions and intravenous hydration for patients with altered consciousness 1
• Avoid medications that cause CNS depression in patients with encephalopathy 1
• Correct any electrolyte imbalances and monitor renal function, especially in patients with pre-existing renal impairment 1

Step 5: Management of Complications

• For severe cases with malignant intracranial hypertension, consider neurosurgical consultation for possible decompressive measures 5
• In rare cases with refractory intracranial hypertension, surgical decompression may be necessary 5
• Ensure access to neuroimaging (MRI and CT) and neurophysiology (EEG) within 24 hours for monitoring 2

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 and consult with relevant specialists 1
• For patients on ECMO with neurological changes, obtain prompt neurological consultation 1
• Be vigilant for PRES during perioperative periods, especially in patients with risk factors 6

Prognosis and Follow-up

• Complete spontaneous remission occurs in most cases without sequelae when managed promptly 1, 7
• Follow-up neuroimaging is recommended to confirm resolution of radiographic findings 8
• The reversibility of clinical and radiologic abnormalities depends on prompt control of blood pressure and/or discontinuation of the offending drug 8

Common Pitfalls to Avoid

• Failure to identify and discontinue the triggering agent can lead to prolonged illness and increased risk of complications 1
• Missing concomitant conditions that may complicate management, such as sepsis or metabolic disturbances 1
• Delayed recognition can result in severe and long-term neurological disability, emphasizing the need for prompt diagnosis and management 6
• Overly rapid correction of blood pressure may lead to cerebral hypoperfusion 1