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

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

Immediately discontinue the offending agent and implement stringent blood pressure control with gradual reduction—these are the two cornerstones of PRES management that directly prevent irreversible neurological injury and death. 1

Step 1: Identify and Remove Triggering Factors

Discontinue the causative agent immediately to prevent further endothelial injury and progression of PRES. 1 Common triggers requiring immediate cessation include:

• Immunosuppressants (particularly cyclosporine) 1
• Chemotherapy and high-dose antineoplastic therapy 1
• Anti-TNF therapy such as infliximab 1
• Any recently initiated or dose-escalated medications 1

The failure to identify and stop the triggering agent leads to prolonged illness and increased risk of irreversible complications. 1 This step takes priority even before blood pressure management in medication-induced cases. 2, 3

Step 2: Blood Pressure Management

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

Critical Blood Pressure Principles:

• Target gradual reduction to avoid cerebral hypoperfusion—abrupt lowering can cause ischemic injury 1, 3
• Monitor blood pressure frequently during the acute phase 1
• Severe hypertension exceeds autoregulatory limits, causing breakthrough brain edema that must be controlled promptly 3
• Delay in blood pressure control may result in death or irreversible neurological sequelae 2

In hemodialysis patients, strict volume control may be a key element for both treatment and prevention of PRES. 4

Step 3: Seizure Management

Administer antiepileptic treatment immediately for patients who develop seizures, as status epilepticus is a life-threatening complication of PRES. 1, 5

Seizure Management Protocol:

• Benzodiazepines for acute seizures: Lorazepam 0.05 mg/kg (maximum 1 mg per dose) IV every 8 hours for symptomatic treatment 1
• Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits to prevent further brain injury 1
• Monitor carefully to avoid CNS depression 1
• Prompt treatment of seizures is essential as delay can result in irreversible sequelae 2

Step 4: Supportive Care Measures

Elevate the head of bed to 30 degrees to reduce intracranial pressure. 1, 6

Additional Supportive Interventions:

• Implement aspiration precautions and IV hydration for patients with altered consciousness 1
• Withhold oral intake and assess swallowing function—substitute all oral medications and nutrition with IV forms if swallowing is impaired 1
• Avoid medications that cause CNS depression in patients with encephalopathy; if sedation is absolutely necessary, use low doses of lorazepam or haloperidol with careful monitoring 1
• Correct electrolyte imbalances and monitor renal function, especially in patients with pre-existing renal impairment 1

Step 5: Diagnostic Confirmation and Monitoring

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

Essential Diagnostic Workup:

• MRI is the gold standard showing T2-weighted or FLAIR hyperintensities in bilateral parietal-occipital lobes, predominantly affecting white matter 1, 7
• CT scan can exclude intracranial hemorrhage when MRI is not immediately feasible 1
• Consider additional workup: fundoscopic exam for papilledema, EEG if seizures are suspected or to rule out non-convulsive status epilepticus, and lumbar puncture with opening pressure if indicated 1

Frequent neurological evaluations and neuroimaging examinations are required for both diagnosis and assessment of the condition. 7

Special Clinical Contexts

PRES Associated with Immunotherapy:

Follow the Society for Immunotherapy of Cancer guidelines for immune-related adverse events. 1

PRES During ECMO:

Neurological consultation is recommended for acute neurological changes. 1

Declining Level of Consciousness:

Patients require urgent ICU assessment for airway protection and ventilatory support. 6 For Grade III-IV encephalopathy, intubate for airway protection and consider propofol for sedation in small doses. 6

Critical Pitfalls to Avoid

• Missing the triggering agent: Failure to discontinue causative medications leads to prolonged illness and complications 1
• Abrupt blood pressure reduction: Can cause cerebral hypoperfusion and ischemic injury—reduction must be gradual 1, 3
• Delayed diagnosis: Early detection is key for rapid recovery and good prognosis; delay may result in death or irreversible neurological sequelae 2, 7
• Missing concomitant conditions: Sepsis, metabolic disturbances, or other complications can worsen outcomes if not addressed 1

Prognosis

Complete spontaneous remission occurs in most cases without sequelae when early detection and management are implemented. 1 The reversibility of clinical and radiologic abnormalities is contingent on prompt blood pressure control and discontinuing the offending drug. 3