Reversible Posterior Leukoencephalopathy Syndrome

Critical Case Conference
by Dr. Darcy Rumberger

Reversible Posterior Leukoencephalopathy Syndrome (RPLS)
— Formerly known as Posterior Reversible Encephalopathy Syndrome (PRES)

Can occur in any age group (children to adults)
Slightly more common in women
Use of cytotoxic and immunosuppressant drugs
Seen in hypertensive encephalopathy and eclampsia as well

4 features:
Headache — can be present for days-weeks
Altered level of consciousness
Visual disturbances — e.g. hemianopsia, neglect, cortical blindness
Seizures

Pathogenesis: unclear — multiple theories: disordered cerebral autoregulation vs endothelial dysfunction vs microinfarction

Diagnosis: No specific diagnostic imaging criteria but imaging suggestive
Often seen on CT but better seen on MRI
Symmetrical white matter edema posterior cerebral hemispheres
Cerebellar and brainstem involvement is common
Not confined to single vascular territory
Resolution on neuroimaging takes days to weeks

Treatment: Anti-hypertensive medication (e.g. nicardipine or esmolol)
Diastolic goal DBP < 100-105
No more than 25% drop in BP to avoid iatrogenic cerebral ischemia
Seizure treatment
Eclampsia — usual management (delivery, magnesium)
Cytotoxic drugs — stop or reduce drug
Treat underlying diseases

Prognosis:
Not always reversible — can have permanent deficits or death although most people recover
Recurrence — rare

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