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

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

Immediate Priority: Identify and Remove the Trigger

The cornerstone of PRES management is immediate identification and discontinuation of the offending agent—whether anticancer therapy, immunosuppressants (particularly cyclosporine), or other precipitating medications—combined with stringent blood pressure control to prevent irreversible neurological injury. 1

Step 1: Discontinue Causative Agents Immediately

• Stop the offending medication without delay, as failure to identify and discontinue the triggering agent leads to prolonged illness and increased risk of permanent complications 1
• Common triggers requiring immediate cessation include:
  • Immunosuppressants, especially cyclosporine 1
  • High-dose antineoplastic therapy 1
  • Anti-TNF therapy such as infliximab 1
  • Any recently initiated or dose-escalated medications 2

Step 2: Implement Stringent Blood Pressure Control

Blood pressure management is the primary therapeutic intervention and must be initiated immediately with very stringent control, targeting gradual reduction to avoid cerebral hypoperfusion. 1

• Reduce blood pressure gradually rather than precipitously to prevent cerebral hypoperfusion, which can worsen outcomes 1, 3
• Monitor blood pressure frequently during the acute phase to ensure it remains within a safe range 1
• The goal is controlled reduction when blood pressure is markedly elevated and cerebral autoregulation has failed 1

Step 3: Manage Seizures Aggressively

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

• For acute seizures, use lorazepam 0.05 mg/kg (maximum 1 mg per dose) IV every 8 hours 1
• Avoid medications that cause excessive CNS depression in patients with encephalopathy 1
• Monitor carefully to balance seizure control against sedation 1

Step 4: Provide Critical Supportive Care

Elevate the head of the bed to 30 degrees to reduce intracranial pressure, implement aspiration precautions, and provide intravenous hydration for patients with altered consciousness. 1, 4

• Withhold oral intake and assess swallowing function before allowing any oral medications or nutrition 1
• Substitute all oral medications with IV forms if swallowing is impaired 1
• For Grade III-IV encephalopathy with declining consciousness, intubate for airway protection 4
• Use propofol in small doses for sedation if mechanical ventilation is required 4

Step 5: Correct Metabolic Derangements

• Correct electrolyte imbalances promptly, particularly in patients with pre-existing renal impairment 1
• Monitor renal function closely, as renal impairment is both a trigger and complicating factor 1
• Follow glucose, potassium, magnesium, and phosphate levels closely 4

Diagnostic Confirmation

Obtain MRI immediately as the gold standard for diagnosis, showing T2-weighted or FLAIR hyperintensities in bilateral parietal-occipital lobes with characteristic vasogenic edema pattern. 1, 2

• MRI is superior to CT for detecting subtle white matter edema and may appear normal on CT in early PRES 2
• Essential MRI sequences include T2-weighted, FLAIR, DWI with ADC maps, and T2* GRE or SWI to detect microhemorrhages 2
• CT can be useful to exclude intracranial hemorrhage when MRI is not immediately available 1, 2

Specialist Consultation and Monitoring

• Obtain neurology consultation for comprehensive neurological assessment and management guidance 1
• Consider additional workup including fundoscopic exam for papilledema, EEG if seizures are suspected or to rule out non-convulsive status epilepticus 1
• Lumbar puncture with opening pressure measurement may be indicated to exclude infectious or inflammatory mimics 1, 2

Special Clinical Scenarios

PRES Associated with Immunotherapy

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

PRES in Systemic Lupus Erythematosus with Refractory Hypertension

• Consider plasmapheresis in addition to multiple antihypertensive therapy when blood pressure remains uncontrolled despite standard measures 5

PRES During ECMO

• Obtain neurological consultation immediately for acute neurological changes 1

Critical Pitfalls to Avoid

• Never delay discontinuation of suspected triggering agents while waiting for diagnostic confirmation, as delay results in death or irreversible neurological sequelae 3
• Do not miss concomitant conditions such as sepsis, metabolic disturbances, or autoimmune disease activation that may complicate management 1
• Avoid excessive sedation in patients with encephalopathy; use short-acting benzodiazepines in small doses only for unmanageable agitation 4
• Do not overlook alternative diagnoses including cerebral amyloid angiopathy-related inflammation, infectious encephalitis, or acute ischemic stroke if clinical course is atypical 2

Expected Prognosis

Complete spontaneous remission occurs in most cases without sequelae when early detection and management are achieved, but the reversibility of clinical and radiologic abnormalities is contingent on prompt blood pressure control and discontinuation of offending drugs 1, 6, 7