Posterior Reversible
Encephalopathy Syndrome
(PRES)
Todd Herrington
Gillian Lieberman, MD
March 2008
Patient RF
„
44-year-old man with a history of metastatic renal cell carcinoma
s/p debulking operation. Now with recurrent mass in left renal bed
and metastasis to abdominal lymph nodes and lungs.
„
Currently on Sutent (multiple RTK inhibitor) and
Gemzar (nucleoside analogue).
„
Presents with headache, fatigue, “word finding difficulties” and
fluctuating mental status including periods of agitation and inability
to recognize family. He is noted to have new onset hypertension
(160s / 100s from a baseline of 130-140s/70-80s).
Differential diagnosis
„
The acute onset of mental status changes has a long differential
diagnosis.
„
In the absence of an obvious cause, several emergent conditions
should be ruled out by head imaging:
„ Hemorrhage
„ Mass effect (midline shift, herniation, increased ICP)
„ Ischemia / infarction
„
Non-contrast CT is the first-line test for assessing intracranial
hemorrhage (seen as hyperdensity) and mass effect…
Patient RF: Baseline and current head CT
8 months ago
present
Bilateral, white-matter
hypodensity with
no mass effect
PACS, BIDMC
Patient RF: Lesions on MRI and CT
MRI (FLAIR)
CT
Lesions are T2hyperintense.
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Patient RF: Multiple lesions on MRI (FLAIR)
Superior
Inferior
*
*
* *
* Central sulcus
Sylvian fissure
*
*
*
Frontal
Temporal
Parietal
Occipital
There are multiple high signal lesions bilaterally in the white matter of all four lobes,
but most predominant in parietal cortex.
PACS, BIDMC
Patient RF: Summary
„
„
Multiple, bilateral, hyperintense
subcortical white-matter lesions
on T2-weighted MRI.
Parietal >> occipital, frontal, and
temporal involvement.
FLAIR
PACS, BIDMC
An abbreviated differential: T2-bright white matter
„
„
„
„
„
„
„
„
„
„
„
„
Neoplastic - glioma, lymphoma, gliomatosis cerebri, metastasis
Vascular - arterial or venous thrombosis, anoxia, vasculitis, amyloid angiopathy
Demyelination - MS, ADEM, acute hemorrhagic encephalomelitis, Schilder’s
disease, Marburg disease, concentric sclerosis
Dysmyelination - leukodystrophies, PKU, MSUD
Infection
Viral - HIV, VZV, JC (PML), measles (SSPE), rubella
Bacterial - Lyme, neurosyphilis
Parasitic - toxoplasmosis
Inflammatory - neurosarcoid, SLE, Behcet’s, Sjogren’s, Wegener’s, polyarteritis
nodosa, scleroderma
Hydrocephalus - early and normal-pressure
Trauma - diffuse axonal injury
Seizure
Toxic - radiation therapy, antineoplastics, immunosuppressants, drugs of
abuse, environmental exposures
Posterior Reversible Encephalopathy Syndrome (PRES)
Other genetic: NF2, Hurler’s syndrome, mytonic dystrophy
Narrowing the differential for patient RF
„
„
„
„
„
Neoplastic - metastatic renal cell cancer
Ischemia/infarction
Infection - PML
Posterior Reversible Encephalopathy Syndrome (PRES)
Toxic - secondary to chemotherapy (Sutent, Gemzar)
Typical appearance of brain metastasis on MRI
„
Most common tumors to metastasize to the brain
(in decreasing frequency): lung, breast, melanoma, renal and colon.
„
Appearance on MRI
„ Multifocal, classically at gray-white junction
„ T1 isotense or mildly hypointense. Hemorrhagic necrosis may
be hyperintense.
„ T2-hyperintense (tumor and surrounding vasogenic edema)
„ Exhibit mass effect, distortion of brain architecture.
„ Enhancing: solid, nodular or irregular ring pattern.
Nonenhancing lesions are less likely to be metastasis.
Companion patients #1-3: Brain metastasis on
post-gadolinium T1W-MRI
Solid enhancing
lesion with
surrounding edema1.
Multiple, ring
enhancing lesions1.
1. www.emedicine.com/Radio/topic101.htm
2. http://www.nature.com/bjc/journal/v89/n2/fig_tab/6601116f1.html
Single enhancing
lesion with central
necrosis,
surrounding edema
and sulcal
effacement2.
Patient RF: C- and C+ MRI
FLAIR
T1, post-gadolinium
Lesions do not enhance.
PACS, BIDMC
Patient RF: Summary
„
„
„
„
Multiple, bilateral, hyperintense,
subcortical white-matter lesions
on T2-weighted MRI.
Parietal >> occipital, frontal, and
temporal involvement.
Mild sulcal effacement.
Nonenhancing.
FLAIR
Post-Gd
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Typical appearance of ischemia and infarction on MRI
„
Diffusion-weighted MRI is sensitive for ischemia within minutes of
the cerebrovascular event, presumably secondary to cytotoxic
edema.
„
Appearance on MRI
„ Lesions confined to a vascular territory, though multiple emboli
can produce multifocal disease.
„ Bright on diffusion-weighted imaging (DWI)
„ Anything that is T2-hyperintense (e.g. cerebral edema) can
falsely elevate signal on DWI (“T2 shine through”).
„ To eliminate the effect of T2 signal, an Apparent Diffusion
Coefficient (ADC) is calculated. Ischemia is dark on ADC
imaging.
Companion patient #4: Right MCA stroke on MRI
DWI
ADC
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Patient RF: possible ischemia on DWI and ADC MRI
DWI
ADC
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Patient RF: Summary
„
„
„
„
„
Multiple, bilateral, hyperintense,
subcortical white-matter lesions
on T2-weighted MRI.
Parietal >> occipital, frontal, and
temporal involvement.
Mild sulcal effacement.
Nonenhancing.
Questionable areas of
ischemia/infarction.
FLAIR
Post-Gd
DWI
ADC
PACS, BIDMC
Infection: PML
„
„
„
„
„
Progressive multifocal leukoencephalopathy - a subacute
demyelinating disorder.
Secondary to reactivation of latent JC virus in the setting of impaired
cell-mediated immunity. (80% AIDS, 13% hematologic malignancy,
5% post-transplant immunosuppression)
Symptoms: commonly weakness, speech disturbance, headache.
Any focal neurologic sign is possible. 10% have seizures.
1-year survival if HIV+: 50% if treated with HAART, 10% if untreated
Appearance on MRI:
„ Multiple T2-hyperintense periventricular and subcortical white
matter lesions.
„ Typically do not enhance or exhibit mass effect.
„ Rarely show diffusion restriction.
Companion patient #5: PML on MRI (FLAIR)
A good fit for patient RF’s imaging, however less likely clinically. PML rarely
seen secondary to anti-neoplastic agents. RF was not leukopenic (WBC 4.1).
www.emedicine.com/radio/topic573.htm
Posterior Reversible Encephalopathy Syndrome
(PRES)
„
„
„
„
Also called Reversible Posterior Leukoencephalopathy Syndrome.
Presents with headache, altered consciousness, visual
disturbances and/or seizures typically in the setting of new-onset
hypertension.
Associated with acute hypertensive encephalopathy, eclampsia and
cytotoxic / immunosuppressive drugs (including Sutent).
Etiology unclear, thought to be secondary to endothelial damage in
the setting of hypertension and failure of cerebrovascular
autoregulation with subsequent vasogenic edema. The posterior
circulation is more sensitive to the effects of hypertension.
Typical appearance of PRES on MRI
„
Appearance on MRI
„ Multifocal T2-hyperintensities
„ Favors parietal and occipital cortex (nearly 100% of cases), but
can involve other cortex, thalamus, basal gaglia, cerebellum and
brainstem.
„ Variable presentation on diffusion weighting imaging. Ischemic
changes on DWI/ADC are associated with worse prognosis.
„ Can exhibit subcortical “gyral” enhancement secondary to
breakdown of the blood-brain barrier.
„ Mass effect associated with edema
Companion patient #6: PRES on MRI (FLAIR)
http://www.mypacs.net/cgi-bin/repos/mpv3_repo/wrm/repo-view.pl?cx_subject=1775907&cx_repo=mpv4_repo
PRES can resolve rapidly
„
Treatment
„ Control hypertension
„ Discontinue cytotoxic drugs
„ Antiepileptics if seizing
„
If treated, most patients exhibit complete neurologic recovery within
~2 weeks accompanied by resolution of the radiologic lesions.
„
Resolution is not always complete. Predictors of poor prognosis
include larger area of involvement and evidence of
ischemia/infarction on MRI.
Companion patient #7: Resolution of PRES on MRI
FLAIR
FLAIR, 6 days later
PRES can resolve rapidly once the causal insult is removed.
PACS, BIDMC
Patient RF’s course
„
Patient RF’s chemotherapy was withheld and his blood pressure
tightly controlled. His symptoms resolved after ~3 days. Repeat
MRI at 5 days was unchanged. He was discharged after 1 week
and has not had a recurrence of symptoms for the last 7 months
despite being restarted on Sutent.
„
He has not had follow-up head imaging.
Patient RF: Summary
„
„
„
„
„
„
Multiple, bilateral, hyperintense
white-matter lesions on T2weighted MRI.
Parietal >> occipital, frontal, and
temporal involvement.
Mild sulcal effacement.
Nonenhancing.
Questionable areas of
ischemia/infarction.
FLAIR
Post-Gd
DWI
ADC
Dx: PRES secondary to
hypertension and/or Sutent toxicity
with possible
secondary ischemia.
PACS, BIDMC
Acknowledgements
A.C. Kim, MD
Gillian Lieberman, MD
Maria Levantakis
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