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

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

The cornerstone of PRES management is immediate identification and removal of the triggering factor, coupled with stringent blood pressure control to prevent further brain injury. 1

Clinical Presentation and Diagnosis

• PRES typically presents with acute neurological deficits including altered consciousness, visual disturbances, headaches, and seizures in patients with various underlying conditions 1
• Diagnosis is confirmed by MRI showing characteristic T2-weighted hyperintensities in bilateral parietal-occipital lobes, predominantly affecting white matter 1, 2
• Common triggers include hypertension, renal impairment, autoimmune diseases, anticancer therapy, transplantation, and immunosuppressive medications (particularly cyclosporine) 1, 3

Management Algorithm

Step 1: Identify and Address Underlying Causes

• Discontinue any offending agent immediately (e.g., anticancer therapy, immunosuppressants) to prevent further endothelial injury 1
• Position patient with head elevated at 30 degrees to help reduce intracranial pressure 4
• Obtain urgent neurological consultation for comprehensive management 4

Step 2: Blood Pressure Management

• Implement stringent blood pressure control with gradual reduction to avoid cerebral hypoperfusion 1
• Monitor blood pressure frequently during the acute phase to ensure it remains within a safe range 1
• When blood pressure is markedly elevated and cerebral autoregulation fails, cerebral edema may develop, especially in posterior brain areas where sympathetic innervation is less pronounced 5

Step 3: Seizure Management

• Administer antiepileptic treatment for patients who develop seizures 1
• Consider prophylactic anticonvulsants in high-risk patients with significant neurological deficits 1
• For acute seizures, benzodiazepines may be used, but monitor carefully to avoid adverse effects 1

Step 4: Supportive Care

• Implement aspiration precautions and intravenous hydration for patients with altered consciousness 1
• Avoid medications that cause CNS depression in patients with encephalopathy 1
• Monitor for and promptly treat infections 4
• Follow glucose, potassium, magnesium, and phosphate levels closely 4
• Consider nutrition through enteral feedings when possible 4

Step 5: Neuroimaging and Monitoring

• MRI is the gold standard for diagnosis and monitoring, but CT 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 4
• Repeat neuroimaging to document resolution of findings 3

Special Considerations

• For PRES associated with immunotherapy, follow the Society for Immunotherapy of Cancer guidelines for immune-related adverse events 1
• For patients requiring ICU care, frequent neurological evaluations are essential as PRES may be the most frequent toxic-metabolic encephalopathy seen in the ICU 6
• In cases with persistent symptoms, consider alternative diagnoses or complications 1

Prognosis and Pitfalls

• Complete spontaneous remission occurs in most cases without sequelae, but early detection and management are key factors for rapid recovery 1, 2
• PRES typically follows a benign course, with visual deficits often resolving as the underlying systemic condition improves 7
• Common pitfalls include:
  • Failure to identify and discontinue the triggering agent 1
  • Missing concomitant conditions that may complicate management (e.g., sepsis, metabolic disturbances) 1
  • Delay in diagnosis and treatment, which may result in death or irreversible neurological sequelae 8