25/11/2014

Posterior Reversible
Encephalopathy
Syndrome in Children:
Is It Really So
Reversible?

Ignacio Delgado; Chae B Whang; Angel SanchezMontanez; Miquel Raspall; Alfons Macaya; Elida Vazquez

Posterior reversible encephalopathy syndrome (PRES)

classically consists on reversible vasogenic edema in the
posterior circulation territories accompanied by
headache, altered mental status, seizures, and visual
disturbance.
Although usually considered benign and reversible,
characteristics of this syndrome in pediatric patients
remain obscure.

Hospital Universitario Vall dHebron, Barcelona, Spain

Electronic adress
adress:: idelgadoalvarez@gmail.com

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A breakdown in cerebral autoregulation results in the

leakage of fluid into the interstitium which is detected as
vasogenic edema.
If promptly recognised and treated, abnormalities can be
completely reversed. If not, evolute to ischemia.
Diffusion-weighted imaging (DWI) can distinguish
vasogenic edema in PRES from cytotoxic edema.
Restricted diffusion have been associated with an adverse
outcome.
Hemorrhage is seen in some patients and prognosis is also
worse.

Covarrubias DJ, et al . Posterior reversible encephalopathy syndrome prognostic utility of quantitative

diffusion-weighted MR images. AJNR Am J Neuroradiol 2002;23;1038-48
Bartynski WS. Posterior Reversible Encephalopathy Syndrome, Part1: Fundamental Imaging and Clinical
Features. AJNR Am J Neuroradiol 2008, 29:1036-42

PRES is becoming increasingly recognized as a common

complication of pediatric cancer therapy.
Risk factors include hypertension (not necessarily acute),
induction chemotherapy, and therapy with
immunosuppressive agents (eg, cyclosporine, tacrolimus,
and corticosteroids).
Blood pressure may be normal in some cases of PRES,
particularly in the settings of chemotherapy,
immunosuppressive therapy, and sepsis
Vazquez E, et al . Side Effects of Oncologic Therapies in the Pediatric Central Nervous System: Update
on Neuroimaging Findings . Radiographics 2011, Vol.31: 1123-1139, 10.1148

Rectrospective analysis

The purpose of this study is to evaluate which

clinical and magnetic resonance imaging
(MRI) findings can help to predict the
prognosis of PRES and whether or not there is
difference between PRES in children and
adults.

Data of the last 7 years (2007

(2007--2013)
pediatric neuroradiology unit of a reference tertiary centre

Twenty children with PRES

* 9 : 11
* 2 to 14 yearsyears-old (7,7 yo mean age)
* Recurrence of PRES in one patient 3 months after
complete recovery from first episode.
episode.

All patients studied by MRI. Follow-up MRI 14/20:

MR imaging (T1WI, T2WI, GET2*, FLAIR and DWI)

Clinical and MRI features and patients outcome

25/11/2014

Most frequent predisposing causes were renal and hematooncologic diseases frequently associated with endotheliotoxic
cytostatic medication:
* cyclosporine A 10/20
tacrolimus 5/20
mycophenolate 3/20
corticoids 3/20
vincristine 2/20.

Parieto-occipital regions were the most commonly

involved in MRI (19/20 [95%]).
Frontal lobe (40%)
Temporal lobe (15%).
9/20 (45%) showed restriction of diffusion (DWI) in
initial MRI
2 patients hemorrhagic changes.

Presenting symptoms
seizures in 85% (17/20)
altered mental status in 65% (13/20)
visual disturbance 25% (5/20)
headache 15%

Follow-up MRI performed on 14 patients (range

period 4-427 days)
6/14 residual lesions (focal laminar necrosis).

Arterial hypertension present in 16/20 (80%) of patients.

(a)
Classical PRES in a transplanted 14yo girl under
antirejection therapy with seizures and headache .
(a) Axial FLAIR MR images demonstrate subcortical
hyperintensities in the posterior circulation territories
(blue arrow) . (b)Axial diffusion-weighted MR image shows
facilitated diffusion consistent with vasogenic edema.

Sequelae
Six patients developed epilepsy
Two patients remained with ataxia
One patient had a persistent mydriasis
One hypotonia
One learning disability
Two patients died after the initial MR examination
but PRES did not play a role in these patients
outcome.
Six of the patients with sequelae had hemorrhage or
DWI lesions in the initial MRI.

(a)

(b)

(b)

(a)

Classical PRES in a transplanted

14yo girl under antirejection therapy
with seizures and headache .
(a) Axial FLAIR MR images
demonstrate subcortical
hypersignals in the posterior
circulation territories (blue arrow) .
(b) Follow-up Axial FLAIR MR
images show resolution of vasogenic
edema after 25 days.
Patient is now asypmptomatic.

(c)

(b)

7yo boy with medulloblastoma

treated with vincristine. (a) Axial
MR images (FLAIR) demonstrate
extensive vasogenic edema in frontal
lobes, parietal and occipital regions
(black arrows) consistent with PRES.
Axial diffusion-weighted MR image
shows restricted diffusion in the left
lesion (red). Follow-up Axial FLAIR
MR image shows gliosis involving the
left occipital lobe (yellow). Patient
remained with epilepsy.

25/11/2014

(a)
(c)

(a)

(b)

(b)

6yo girl with leukemia . (a) Axial MR images (FLAIR)

demonstrate vasogenic edema in posterior parietal area (blue
arrow) . (b)Axial diffusion-weighted MR image shows
restricted diffusion in the lesion (red). (c)Follow-up Axial
FLAIR MR image shows gliosis and atrophy (yellow) .

(a)

(c)

(c)

(d)

4yo boy with leukemia treated

with cyclosporine. (a) Axial
FLAIR MR images show
vasogenic edema in parietal
lobes (blue arrows) consistent
with PRES. (b) Axial GET2*
MR image shows hemorrhagic
changes in the left parietal
lesion (red). (c) Follow-up
Axial FLAIR MR image shows
gliosis involving the left
posterior parietal lobe (yellow)
and hemosiderin deposition
(black
black) Patient remained with
seizures.

(b)
Recurrent PRES in a 3yo boy
after renal transplantation.
(a) Axial FLAIR MR images
show focal hyperintensities in
right frontal and parietal lobes
(blue arrow) . (b) 4 day followup MR image shows partial
recovery. (c) Axial FLAIR MR
images 3 months later with new
similar lesions in contralateral
parietal lobe (red) (d) 1 year
follow-up with total resolution.

(d)

7yo boy with PRES. (a) Vasogenic

edema involving frontal an parietal
lobes in axial FLAIR MR images (blue
arrow). (b) Follow-up MRI shows total
recovering . (c) Initial coronal DWI
with restriction in right hypoccampus
and thalamus (red). (d) Patient
developed messial sclerosis (yellow)

(a)

(b)

6yo girl with PRES . (a) Axial FLAIR MR images shows

edema in occipitotemporal lobe (blue arrow) .
(b)Axial diffusion-weighted MR image shows restricted
diffusion (red). ASL perfusion in acute (c) and follow-up
(d). Note the focal hyperperfusion during the acute phase
and hypoperfusion 3 months later.
(c)

(d)

Posterior reversible encephalopathy may develop in

patients who have renal insufficiency or hypertension
or who are immunosuppressed. This syndrome
should be recognized immediately and trigger agents
must be discontinued to prevent long-term sequelae.
Recurrence of PRES is infrequent.
PRES has not a so good long-term prognosis in
children. Epilepsy is the most frequent sequela.
Diffusion-weighted imaging restriction is a sign of
cytotoxic non reversible edema and indicate poor
prognosis. The presence of hemorrhagic changes in
the initial MRI is also linked with a worse patients
outcome.

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MRI may help predicting the prognosis of PRES.

PRES in children is not only linked with
hypertension like in adult population but also with
chemotherapy, immunosupressors and other
endotheliotoxic medication, which play and
important role in the its pathophysiology .
Early recognition of PRES, discontinuation of
trigger chemoterapy agent and hypertension control
is crucial to avoid sequelae.