What is the management of Posterior Reversible Encephalopathy Syndrome (PRES)?

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

The immediate management of PRES requires discontinuation of the offending agent and stringent blood pressure control with gradual reduction to avoid cerebral hypoperfusion, combined with antiepileptic treatment for seizures. 1

Step 1: Identify and Remove Triggering Factors

Immediately discontinue the causative agent to prevent further endothelial injury and progression of PRES. 1 Common triggers include:

• Immunosuppressants (particularly cyclosporine) 1
• High-dose chemotherapy and antineoplastic agents 1
• Anti-TNF therapy such as infliximab 1
• Allogenic stem-cell or solid organ transplantation 1

A critical pitfall is failure to identify and stop the triggering medication, which leads to prolonged illness and increased complication risk. 1

Step 2: Blood Pressure Management

Implement very stringent blood pressure control as the cornerstone of therapy, with gradual reduction to prevent cerebral hypoperfusion. 1 The European Society of Cardiology emphasizes this as the primary therapeutic intervention, especially when blood pressure is markedly elevated and cerebral autoregulation has failed. 1

• Monitor blood pressure frequently during the acute phase 1
• Avoid rapid reduction that could cause cerebral hypoperfusion 1, 2
• Target gradual normalization rather than aggressive lowering 1

Step 3: Seizure Management

Administer antiepileptic treatment for patients who develop seizures, and consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits. 1

• For acute seizures: lorazepam 0.05 mg/kg (maximum 1 mg per dose) IV every 8 hours 1
• Monitor carefully to avoid CNS depression 1
• Treatment of seizures or status epilepticus is essential, as delay may result in irreversible neurological sequelae or death 3

Step 4: Supportive Care Measures

Elevate the head of the bed to 30 degrees to reduce intracranial pressure. 1

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

• Withhold oral intake and assess swallowing function 1
• Substitute all oral medications and nutrition with IV forms if swallowing is impaired 1
• Avoid medications that cause CNS depression in patients with encephalopathy 1
• Use low doses of lorazepam or haloperidol only when necessary, with careful monitoring 1

Step 5: Electrolyte and Metabolic Management

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

Step 6: Diagnostic Confirmation and Monitoring

Obtain neurology consultation for comprehensive neurological assessment and management guidance. 1

MRI is the gold standard for diagnosis, showing T2-weighted or FLAIR hyperintensities in bilateral parietal-occipital lobes, predominantly affecting white matter. 1, 2, 3 CT scan can be useful to exclude intracranial hemorrhage when MRI is not immediately feasible. 1

Consider additional diagnostic workup:

• Fundoscopic exam to assess for papilledema 1
• EEG if seizures are suspected or to rule out non-convulsive status epilepticus 1
• Lumbar puncture with opening pressure measurement if indicated 1

Special Clinical Contexts

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

For PRES during ECMO, neurological consultation is recommended for acute neurological changes. 1

For patients with declining level of consciousness, urgent assessment by ICU staff for airway protection and ventilatory support is required. 4

Common Pitfalls to Avoid

• Missing concomitant conditions such as sepsis or metabolic disturbances that may complicate management 1
• Delaying diagnosis and treatment, which may result in death or irreversible neurological sequelae 3
• Failing to consider alternative diagnoses when symptoms persist, including cerebral amyloid angiopathy, subacute infarcts, or infectious encephalitis 5

Prognosis

Complete spontaneous remission occurs in most cases without sequelae, but early detection and management are key factors for rapid recovery and good outcomes. 1, 6 The reversibility of clinical and radiologic abnormalities is contingent on prompt blood pressure control and discontinuation of the offending drug. 2