Differential Diagnoses of PRES in Transplanted Children

PRESCNS infectionsNeoplastic (PTLD)StrokePML
Type of transplantationAllAllAllIschemic: heart > other SOT > HSCTAll
Hemorrhagic: HSCT > SOT
Timing after transplantationMostly <100 d in HSCTBacterial: <30 dUsually delayedPerioperative or laterDelayed (>6 mo)
May be delayed in SOTViral and others: >30 d
Type of onsetAcuteAcute/subacuteSubacuteAcuteSubacute
Presenting signs/symptomsSeizures (often nonconvulsive and SE)Mental status changesMental status changesFocal neurologic signsMental status changes
Visual symptomsFeverHeadacheSeizures (often hemiclonic)Focal neurologic signs
Mental status changesSeizures
Location/patternSubcortical WM/cortical, usually bilateral posterior lobes ± other lobes ± brainstem and cerebellumWM focal, multifocal, or diffuse ± cortical (depending on the type of microorganism)WM focal or multifocal masses, meningesIschemic: unilateral WM/corticalWM multifocal
Hemorrhagic: unilateral bleeding
 CT scanNormal or low-densityNormal or low-densityNormal or low-densityLow-density or bleedingNormal or low-density
 Conventional MRIHigh T2 signalHigh T2 signalLow or high T2 signalHigh T2 signalHigh T2 signal
 ContrastNonenhancingEnhancingPeripheral enhancingNonenhancingNonenhancing
Laboratory findingsOften hypomagnesemiaBlood cultures and PCR sometimes diagnosticOften not significantIschemic: not significantNot significant
Hemorrhagic: bleeding diathesis
CSF findingsNot significantOften diagnosticRarely diagnostic (cytology)Not significantPCR for JCV often diagnostic
EEG featuresRhythmic spikes (NCSE), PLEDs, and/or slowing in the posterior regionsDiffuse or focal slowingSometimes focal slowingUnilateral PLEDs and/or slowingSometimes focal slowing
  • CSF, cerebrospinal fluid; JCV, John Cunningham Poliomavirus; NCSE, nonconvulsive status epilepticus; PCR, polymerase chain reaction; PLED, periodic lateralized epileptiform discharge; PML, progressive multifocal leukoencephalopathy; PTLD, posttransplant lymphoproliferative disease; WM, white matter.