What is the treatment for posterior reversible encephalopathy syndrome (PRES)?

Treatment of Posterior Reversible Encephalopathy Syndrome (PRES)

The treatment of PRES centers on immediate discontinuation of any offending agent and aggressive blood pressure control with gradual reduction to avoid cerebral hypoperfusion, which leads to complete recovery in most cases without neurological sequelae. 1

Immediate Management Algorithm

Step 1: Identify and Remove Triggering Factors

• Immediately discontinue the causative agent such as immunosuppressants (particularly cyclosporine), chemotherapy agents (including ifosfamide and infliximab), or any recently initiated medications that may have precipitated the syndrome 1, 2
• Common triggers requiring immediate cessation include anticancer therapy, immunosuppressive medications, and biologics used in autoimmune conditions 1

Step 2: Blood Pressure Management (Cornerstone of Treatment)

• Implement very stringent blood pressure control as the primary therapeutic intervention, targeting gradual reduction rather than rapid normalization to prevent cerebral hypoperfusion 1
• The European Society of Cardiology emphasizes gradual reduction when blood pressure is markedly elevated and cerebral autoregulation has failed 1
• Monitor blood pressure frequently during the acute phase, checking every 4-6 hours until stabilization 1
• Critical pitfall to avoid: Overly aggressive blood pressure reduction can cause cerebral ischemia; the goal is controlled, gradual lowering 1

Step 3: Seizure Management

• Administer antiepileptic medications for patients who develop seizures 1, 3
• Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits to prevent further brain injury 1
• Use benzodiazepines for acute symptomatic seizure control, monitoring carefully for respiratory depression 1

Step 4: Supportive Care Measures

• Implement aspiration precautions and provide intravenous hydration for patients with altered consciousness 1
• Avoid medications that cause CNS depression in patients with encephalopathy 1
• Correct electrolyte imbalances if present, particularly hyponatremia, and monitor renal function closely 1

Severe Cases Requiring Intensive Management

Malignant PRES Recognition

• A minority of patients develop "malignant PRES" which can be fatal and requires ICU-level care 3
• Transfer to ICU for Grade III-IV encephalopathy (severe somnolence, coma, or seizures) 4
• Maintain airway protection; consider intubation for patients with significantly altered mental status 4

Advanced Interventions for Refractory Cases

• Hyperosmolar therapy may be required for refractory intracranial hypertension 3
• Ventriculostomy placement for monitoring and management of elevated intracranial pressure 3
• Emergent decompressive craniectomy may be necessary in rare cases with malignant intracranial hypertension unresponsive to medical management 5

Special Clinical Contexts

PRES Associated with Immunotherapy

• Follow the Society for Immunotherapy of Cancer guidelines for immune-related adverse events when PRES occurs in the context of checkpoint inhibitors or other immunotherapies 1

PRES During ECMO

• Obtain immediate neurological consultation for acute neurological changes in patients on extracorporeal membrane oxygenation 1

Post-Neurosurgical PRES

• PRES can occur after posterior fossa tumor resection, particularly when tumors compress the brainstem 6
• Rapid diagnosis and aggressive management with antihypertensive and anticonvulsant medications are essential for achieving complete recovery 6

Monitoring During Recovery

• Obtain neurological assessments every 4-6 hours until complete resolution of symptoms 4
• Most patients recover completely within 3-5 days without neurological sequelae when managed appropriately 4
• Complete spontaneous remission occurs in most cases, but early detection and prompt management are key factors for rapid recovery and good outcomes 1

Critical Pitfalls to Avoid

• Failure to identify and discontinue the triggering agent leads to prolonged illness and increased risk of permanent complications 1
• Missing concomitant conditions such as sepsis, metabolic disturbances, or electrolyte abnormalities that may complicate management 1
• Overly aggressive blood pressure reduction causing cerebral hypoperfusion and ischemic injury 1
• Delaying treatment while pursuing extensive diagnostic workup; treatment should begin immediately upon clinical suspicion 2

Prognosis

• The reversibility of both clinical and radiologic abnormalities is contingent on prompt blood pressure control and discontinuation of offending drugs 2
• Less commonly, patients may have residual neurologic sequelae, particularly in cases with associated structural injury such as intracranial hemorrhage or stroke 3
• Early aggressive management prevents permanent neurological deficits even in severe cases requiring surgical intervention 5, 6