Pediatric Posterior Reversible Encephalopathy Syndrome

  • Etiology: Cerebrovascular autoregulatory disorder where hypertension and failed autoregulation leads to hyperperfusion which leads to endothelial dysfunction which leads to increased interstitial fluid and brain parenchymal vasogenic edema
  • Imaging CT: Not sensitive to detecting PRES
  • Imaging MRI:
    — Classically affects occipital and parietal regions relating to posterior cerebral artery supply
    — Characteristic imaging findings are subcortical or cortical vasogenic edema with or without diffusion restriction with or without hemorrhage – typically posterior and reversible
    — Note: Not always posterior, not always reversible (typically has vasogenic edema (reversible) but occasionally has cytotoxic edema (irreversible))
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — Risk factors: acute or subacute systemic hypertension, drug toxicity in particular: chemotherapy (cyclosporine, cisplatin, tacrolimus), immune suppression, severe infection, uremia, eclampsia or pre-eclampsia, thrombotic microangiopathies (DIC, TTP)
    — Acute or subacute presentation – altered mental status (60%) or headache (54%) or seizure (50%) or visual symptoms (36%) – cortical blindness or focal neurological defect (19%)
    — Formula for diagnosis is clinical suspicion – Risk factors and acute or subacute presentation and characteristic imaging findings

Radiology Cases of Posterior Reversible Encephalopathy Syndrome

MRI of posterior reversible encephalopathy syndrome / PRES
Axial FLAIR MRI without contrast of the brain shows bilaterally multiple areas of hyperintense signal in the cortex and subcortical white matter.