Management of Posterior Reversible Encephalopathy Syndrome (PRES)
The cornerstone of PRES management is immediate identification and removal of the triggering factor, along with stringent blood pressure control to prevent further brain injury. 1
Step 1: Identify and Address Underlying Causes
- Immediately discontinue any potential offending agents such as anticancer therapy, immunosuppressants (particularly cyclosporine), or other medications that may have triggered PRES 1
- Common triggers include pre-existing hypertension, renal impairment, autoimmune diseases, high-dose antineoplastic therapy, stem-cell transplantation, solid organ transplantation, and immunosuppression 1, 2
- For medication-related PRES (e.g., from infliximab or other biologics), prompt cessation of the causative agent is essential 1
Step 2: Blood Pressure Management
- Implement strict blood pressure control with gradual reduction to avoid cerebral hypoperfusion 1, 3
- Target a controlled reduction rather than rapid normalization to prevent complications from cerebral hypoperfusion 1
- Monitor blood pressure frequently during the acute phase to ensure it remains within a safe range 1
Step 3: Seizure Management
- Administer antiepileptic treatment for patients who develop seizures 1, 3
- Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits 1
- Benzodiazepines (e.g., midazolam) may be used for acute seizure management 4
Step 4: Supportive Care
- Position patients with head elevated at 30 degrees to help reduce intracranial pressure 5
- For patients with declining consciousness, obtain urgent ICU assessment for airway protection and ventilatory support 5
- Implement aspiration precautions and intravenous hydration for patients with altered consciousness 1
- Avoid medications that cause CNS depression in patients with encephalopathy 1
Step 5: Electrolyte and Fluid Management
- Correct any electrolyte imbalances and monitor renal function, especially in patients with pre-existing renal impairment 1
- Consider diuretics like furosemide to reduce cerebral edema in appropriate cases 4
- Administer dexamethasone in cases with significant cerebral edema 4
Diagnostic Considerations
- MRI is the gold standard for diagnosis, showing increased signal intensity on T2-weighted or FLAIR imaging in posterior brain regions 1, 6
- CT scan can be useful to exclude intracranial hemorrhage when MRI is not feasible 1
- Ensure access to neuroimaging (MRI and CT) and neurophysiology (EEG) within 24 hours 5
Special Populations
- For PRES associated with immunotherapy, follow the Society for Immunotherapy of Cancer guidelines for immune-related adverse events 1
- In cases of PRES during ECMO, obtain prompt neurological consultation for acute neurological changes 1
- For PRES in SLE patients, consider aggressive immunosuppressive therapy (cyclophosphamide) and plasmapheresis in severe cases 2
Monitoring and Follow-up
- Perform frequent neurological assessments to monitor for improvement or deterioration 5, 7
- Repeat neuroimaging to document resolution of radiological abnormalities 6
- Monitor for and promptly treat infections, and follow glucose and electrolyte levels closely 5
Prognosis
- Complete spontaneous remission occurs in most cases without sequelae when managed promptly 1, 7
- Early detection and management are key factors for rapid recovery and good outcomes 7
- Delayed diagnosis and improper management may result in permanent brain injury or neurological sequelae 4
Common Pitfalls to Avoid
- Failure to identify and discontinue the triggering agent can lead to prolonged illness and increased risk of complications 1
- Missing concomitant conditions that may complicate management, such as sepsis or metabolic disturbances 1
- Overly rapid correction of blood pressure may lead to cerebral hypoperfusion 1, 3