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

Management of Atypical Posterior Reversible Encephalopathy Syndrome (PRES)

The management of atypical PRES requires immediate identification and removal of triggering factors, very stringent blood pressure control, and aggressive treatment of cerebral edema to prevent permanent neurological sequelae. 1

Initial Assessment and Stabilization

• Identify and address triggering factors:

  • Discontinue offending medications (immunosuppressants, chemotherapy agents) 1
  • Immediate delivery for eclampsia cases 1
  • Treat underlying autoimmune conditions 1
  • Address renal impairment/failure 1

• Blood pressure management:

  • Implement very stringent blood pressure control with gradual reduction to avoid cerebral hypoperfusion 1
  • For severe hypertension (systolic BP ≥220 mmHg), use IV antihypertensives:
    • First-line: IV labetalol
    • Oral option: nifedipine
    • Second-line: IV hydralazine 1

Management of Cerebral Edema and Increased Intracranial Pressure

• Position patient with head elevated at 30 degrees to decrease hydrostatic pressure and cerebral edema 1, 2

• Hyperosmolar therapy for evidence of cerebral edema or increased intracranial pressure:

  • Mannitol: 0.5-1 g/kg IV initially, then 0.25-1 g/kg every 6 hours for maintenance 1
  • OR Hypertonic 3% saline: 5 ml/kg IV over 15 minutes initially, then 1 ml/kg/hour IV to target serum sodium 150-155 meq/L 1

• Consider hyperventilation to achieve PaCO2 of 30-40 mmHg for acute management of intracranial hypertension 1

• For refractory intracranial hypertension:

  • Consider decompressive craniectomy in severe cases with malignant cerebral edema 3, 2
  • Decision for decompressive craniectomy should be based on risk-benefit discussion between the multidisciplinary medical team and patient surrogate 2

Seizure Management

• For active seizures:
  • Administer antiepileptic treatment promptly 1
  • Benzodiazepines for acute seizure control 1
  • Consider prophylactic anticonvulsants in high-risk patients 1
  • Immediate treatment of seizures is required 2

Monitoring and Supportive Care

• Neurological monitoring:

  • Frequent neurological evaluations for signs of elevated intracranial pressure 2
  • Monitor electrolytes every 4-6 hours 1
  • Consider neurological consultation for acute neurological changes 2

• Imaging:

  • Brain CT to rule out other causes of decreased mental status 2
  • Follow-up MRI to assess resolution of lesions 4

• Additional supportive measures:

  • For severe encephalopathy (Grade III/IV), consider intubation for airway protection 2
  • Avoid stimulation and unnecessary sedation if possible 2
  • Surveillance for and prompt treatment of infections 2

Special Considerations for Atypical PRES

• Recognize atypical imaging patterns:

  • Lesions may involve cerebellum (59.5%), basal ganglia (24.3%), periventricular/deep white matter (22.5%), pons (22.3%), brainstem (20.7%), and thalamus (20.5%) 4
  • Atypical presentations may mimic other neurological conditions 5

• Be aware of potential for recurrence:

  • PRES can recur, especially in patients with end-stage renal disease or poorly controlled hypertension 6, 7
  • Regular monitoring of patients on immunosuppressive therapy is important for early detection of recurrent PRES 1

Prognosis and Follow-up

• With appropriate management, symptoms and radiological findings typically resolve within 2-3 weeks 4

• Poor prognostic factors:

  • Altered sensorium
  • Extensive cerebral edema
  • Presence of hemorrhage on imaging
  • Delayed control of causative factors 1

• The decision to reintroduce prior anticancer therapy must be individualized and carefully considered in cases of chemotherapy-induced PRES 1

Pitfalls and Caveats

• Delayed diagnosis and treatment may lead to permanent neurological sequelae 1
• Atypical PRES may present with severe and prolonged impairment of consciousness or unusual imaging patterns, making diagnosis challenging 5
• Careful systemic anticoagulation monitoring and resumption after decompressive craniectomy is essential if surgical intervention is required 2