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 discontinuation of the offending agent combined with stringent blood pressure control, targeting gradual reduction to avoid cerebral hypoperfusion, which leads to complete spontaneous remission in most cases without sequelae. 1

Immediate Actions: Identify and Remove Triggers

Discontinue the causative agent immediately - this is the most critical first step that directly impacts reversibility of the syndrome. 1, 2

Common triggers requiring immediate cessation include:

• Immunosuppressive medications (particularly cyclosporine) 1
• Chemotherapy agents 1
• Anti-TNF therapies (such as infliximab) 1
• Immunotherapy agents 1

Underlying conditions that precipitate PRES include:

• Pre-existing arterial hypertension 1
• Renal impairment 1, 3
• Autoimmune diseases 1, 3
• Allogenic stem-cell or solid organ transplantation 1
• Eclampsia 3

Blood Pressure Management: The Primary Therapeutic Intervention

Implement very stringent blood pressure control with gradual reduction - this is the primary therapeutic intervention recommended by the European Society of Cardiology. 1 The goal is to reduce blood pressure without causing cerebral hypoperfusion, which can worsen outcomes. 1, 2

Specific antihypertensive agents mentioned in the literature:

• Labetalol for controlled reduction 4
• Nitroprusside for hypertensive emergencies 4
• Nifedipine with furosemide for acute management 4

Monitor blood pressure frequently during the acute phase to ensure it remains within a safe range and prevent complications. 1

Seizure Management

Administer antiepileptic treatment for patients who develop seizures, with phenytoin being specifically mentioned as an effective agent. 4

Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits to prevent further brain injury. 1

For acute seizure management:

• Benzodiazepines (such as lorazepam 0.05 mg/kg, maximum 1 mg per dose IV every 8 hours) can be used for symptomatic treatment, but monitor carefully to avoid CNS depression. 5, 1

Supportive Care Measures

Elevate the head of the bed to 30 degrees to help reduce intracranial pressure. 6

Implement aspiration precautions and intravenous hydration for patients with altered consciousness. 5, 1

Avoid medications that cause CNS depression in patients with encephalopathy. 5, 1

For agitated patients requiring sedation:

• Use low doses of lorazepam (0.05 mg/kg, maximum 1 mg per dose IV every 8 hours) or haloperidol (0.05 mg/kg, maximum 1 mg per dose IV every 6 hours) with careful monitoring. 5

Withhold oral intake and assess swallowing function - substitute all oral medications and nutrition with IV forms if swallowing is impaired. 5

Electrolyte and Metabolic Management

Correct electrolyte imbalances if present and monitor renal function closely, especially in patients with pre-existing renal impairment. 1

Diagnostic Confirmation and Monitoring

MRI is the gold standard for diagnosis, showing increased signal intensity on T2-weighted or FLAIR imaging in posterior brain regions (bilateral parietal-occipital lobes with predominantly white matter involvement). 1, 7, 3

CT scan can be useful to exclude intracranial hemorrhage when MRI is not immediately feasible. 1, 7

Obtain neurology consultation for comprehensive neurological assessment and management guidance. 5

Consider additional diagnostic workup:

• Fundoscopic exam to assess for papilledema 5
• EEG if seizures are suspected or to rule out non-convulsive status epilepticus 5
• Lumbar puncture with opening pressure measurement if indicated 5

Special Populations and Contexts

For PRES associated with immunotherapy, follow the Society for Immunotherapy of Cancer guidelines for immune-related adverse events. 1

For eclampsia cases, consider induction of vaginal delivery as part of the management strategy. 4

For patients requiring ICU-level care with declining consciousness, urgent assessment for airway protection and ventilatory support is necessary. 6

Anti-Edema Therapy

Mannitol (20%, 60 mL every 3 hours) can be administered for cerebral edema management in severe cases. 4

Common Pitfalls to Avoid

Failure to identify and discontinue the triggering agent is the most critical error - this leads to prolonged illness and increased risk of permanent neurological damage. 1

Missing concomitant conditions such as sepsis, metabolic disturbances, or infections that may complicate management can lead to poor outcomes. 1

Delaying recognition and treatment - early detection is key for rapid recovery and good prognosis, as the reversibility of clinical and radiologic abnormalities is contingent on prompt intervention. 1, 2, 3

Over-aggressive blood pressure reduction can cause cerebral hypoperfusion and worsen outcomes - reduction must be gradual. 1

Prognosis and Follow-up

Complete spontaneous remission occurs in most cases without sequelae when managed appropriately. 1 Clinical improvement typically begins within days, with normalization of neurological findings often occurring within 7-15 days. 4, 8

Repeat neuroimaging should be performed to document resolution of vasogenic edema and confirm diagnosis. 2, 4

Be aware that PRES can be recurrent in some individuals, particularly those with persistent risk factors such as end-stage renal disease requiring ongoing vigilance. 8