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

Management of Posterior Reversible Encephalopathy Syndrome (PRES)

The management of PRES requires immediate cessation of triggering factors, very stringent blood pressure control, and symptomatic treatment of seizures to prevent permanent neurological damage. 1

Clinical Presentation and Diagnosis

PRES typically presents with:

• Acute neurological deficits including altered consciousness
• Visual disturbances or blindness
• Headaches
• Seizures

Diagnosis is confirmed by:

• Clinical presentation with risk factors
• T2-weighted MRI showing hyperintensities predominantly in bilateral parieto-occipital lobes (vasogenic edema)
• Although classically posterior, atypical presentations may involve frontal regions, cerebellum, brainstem, and deep white matter 2

Management Algorithm

1. Immediate Interventions

• Identify and remove triggering factors 1

  • Discontinue offending medications (immunosuppressants, chemotherapy, etc.)
  • Immediate delivery for eclampsia cases
  • Address underlying autoimmune conditions

• Blood pressure control

  • Very stringent control is crucial 1
  • Target gradual reduction to avoid cerebral hypoperfusion
  • IV antihypertensives for severe hypertension

• Seizure management

  • Administer antiepileptic treatment for active seizures 1
  • Benzodiazepines for acute seizure control
  • Consider prophylactic anticonvulsants in high-risk patients

2. Supportive Care

• Correction of electrolyte imbalances 1
• Monitor neurological status closely
• ICU monitoring for severe cases 3

3. Specific Management Based on Etiology

• Medication-induced PRES

  • Discontinue offending agents (cyclosporin, chemotherapy) 1
  • Individual assessment for reintroduction after resolution

• Hypertension-related PRES

  • Gradual blood pressure reduction to avoid cerebral hypoperfusion 1
  • Address underlying causes of hypertension (e.g., renal artery stenosis) 4

• Autoimmune-related PRES

  • Treat underlying autoimmune condition
  • Consider immunosuppressive therapy adjustment

Monitoring and Follow-up

• Regular neurological assessments
• Follow-up MRI to confirm resolution of vasogenic edema
• Monitor for potential complications:
  • Cerebral ischemia
  • Intracranial hemorrhage
  • Permanent neurological deficits

Prognosis

PRES is usually reversible with appropriate supportive management within 2 weeks 1. However, delayed diagnosis and treatment may lead to permanent neurological sequelae 3. Factors associated with poor outcomes include:

• Altered sensorium
• Extensive cerebral edema
• Presence of hemorrhage on imaging
• Delayed control of causative factors 5

Important Caveats and Pitfalls

• "Reversible" is not always true: While most cases resolve completely, permanent neurological damage can occur in 10-20% of cases 2
• "Posterior" is not exclusive: Up to 64% of patients may have frontal involvement and 43% may have cerebellar involvement 2
• Beware of complications: Some patients may develop ischemic or hemorrhagic lesions 2
• Reintroduction of causative agents: Decision to reintroduce prior anticancer therapy must be individualized and carefully considered 1
• Differential diagnosis: Important to distinguish from other causes of acute encephalopathy, stroke, or CNS infection

With prompt recognition and appropriate management, most patients with PRES recover fully without long-term neurological sequelae.