Wednesday, August 1, 2012

Posterior Reversible Encephalopathy Syndrome

Today in Morning Report, we talked about Posterior Reversible Encephalopathy Syndrome (PRES), which is also known under the moniker, Reversible Posterior Leukoencephalopathy Syndrome (RPLS).


PRES is a neurologic syndrome diagnosed by clinical AND radiologic findings:

Clinical Presentation
Classically characterized by the following:
- headaches
- altered level of consciousness
- visual changes
- seizures

Radiologic Findings
The classic findings upon neuroimaging of PRES is symmetrical white matter edema in the posterior cerebral hemispheres, particularly the parieto-occipital regions, although this not always the case. PRES can often been seen on CT imaging of the head, although MRI brain is best.

Here's a CT head non-contrast revealing bilateral hypodensities involving the occipo-parietal region.


Here's a  T2-weighted MRI brain demonstrating multiple cortico-subcortical areas hyperintense lesions involving the occipital and parietal lobes, as well as the pons.

   
Causes of PRES
The most common causes of PRES include:
- hypertensive encephalopathy
- eclampsia
- immunosupprseive medications, including calcineurin inhibitors (cyclosporine, tacrolimus)
- TTP/HUS
- vasculitis (e.g. SLE, PAN)
- renal failure (acute and/or chronic)

Management of PRES
- The most important aspect of management is to treat the underlying cause (whether this means blood pressure lowering, delivery of baby, discontinuation of offending medication, plasmapheresis, etc.)! 
- Seizures can be treated with antiepileptic agents, most commonly phenytoin. 

Most patients demonstrate the "R" of PRES, meaning their symptoms are reversed by treating the underlying cause, often with two weeks of management, although a small minority do not make a full neurologic recovery.