Reversible posterior leukoencephalopathy syndrome

MRI FLAIR sequence with axial section showing cortical hyperintensity in bilateral parietal/occipital, subcortical white matter

MRI FLAIR sequence with axial section showing cortical hyperintensity in bilateral parietal/occipital, subcortical white matter

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical radiographic syndrome of different etiologies that are grouped together because of similar neuroimaging findings. These include: PRES or posterior reversible encephalopathy syndrome, reversible posterior cerebral edema syndrome, & brain capillary leak syndrome. 

Illness script: In patients with hypertensive crisis, preeclampsia, or on cytotoxic immunosuppressive therapy, reversible posterior leukoencephalopathy syndrome (RPLS) may result from disordered cerebral autoregulation and endothelial dysfunction leading to vasogenic edema—giving rise to a clinical syndrome of headache, confusion, visual symptoms, and seizures, often with recovery in two weeks but with potential for mortality due to increased intracranial pressure if not treated. 

Management consists of: 

  • Blood pressure management with an easily titratable parenteral agent like labetalol or nicardipine
  • Withdrawing or lowering the dose of the offending cytotoxic agent/immunosuppressant if able
  • Antiepileptic drugs, preferably parenteral, if seizures occur--although these can be discontinued when imaging findings resolved as seizures following this are rare