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EE-52: Unilateral Posterior
Reversible Encephalopathy
Syndrome (UPRES) in a Patient
with Sickle Cell Disease
Yankai Sun, MD, Shalabh Bobra, MD, Hasit Mehta, MD, Michael
Tenner, MD, Brian Rigney, MD, Amrita Arneja, MD
Department of Radiology, Westchester Medical Center
New York Medical College, Valhalla, New York
Disclosure
• Authors have no disclosure.
Purpose
• To discuss the presentation, pathophysiology
and imaging appearance of posterior
reversible encephalopathy syndrome
(PRES).
• To illustrate the imaging appearance of an
unusual unilateral variant of posterior
reversible encephalopathy syndrome
(UPRES) in a patient with sickle cell disease.
Purpose
• Posterior reversible encephalopathy syndrome
(PRES)
– A reversible neurologic syndrome
– Variety of presenting symptoms and causes.
• Typical presenting symptoms of PRES
– Headache, seizure, altered mental status or vision loss.
• Common causes of PRES
– Hypertension, pre-eclampsia/eclampsia,
immunosuppressive therapy, severe hypercalcemia, or
thrombocytopenic syndromes.
Purpose
Classic PRES. Axial T2weighted FLAIR image of a
different patient which
demonstrates subcortical
signal hyperintensity in the
bilateral parieto-occipital
lobes.
• Imaging appearance
– Classic PRES shows signal hyperintensity in the bilateral parietooccipital and/or posterior frontal cortical and subcortical white matter on
FLAIR (fluid-attenuated inversion recovery) images.
Purpose
• Imaging appearance (cont.)
– Diffusion-weighted imaging (DWI)
• More than 50% show edema isointense to normal appearing
parenchyma.
• 28% may have “T2 shine-through” hyperintensity on FLAIR and DWI.
• Less than 20% may show restricted diffusion.
– Atypical involvement includes the brainstem, basal ganglia,
cerebellum, or the unilateral variant.
• Pathophysiology of PRES
– Not entirely clear but widely discussed hypothesis includes:
• Hyper-perfusion state with blood brain barrier and endothelial damage
resulting in capillary leakage and subsequent cortical and subcortical
edema.
Purpose
• Pathophysiology of PRES (cont.)
– PRES favors the posterior cerebral circulation.
– Relative lack of arteriole sympathetic innervation in the posterior
circulation.
– Sympathetic innervation presumably protects marked increased
intravascular pressure.
– Disruption of blood brain barrier and endothelial damage lead to
loss of autoregulatory capability.
• Atypical locations of PRES
–
–
–
–
Brainstem
Basal ganglia
Cerebellum
Unilateral variant
• UPRES only accounts for 2-3% of all patients with PRES.
Purpose
• Sickle cell disease (SCD)
–
–
–
–
–
Autosomal recessive
Defect in the beta-chain hemoglobin
Microvascular occlusion
Bone infarct and osteomyelitis
Moyamoya disease
• Cerebrovascular involvement in patients with SCD
– Cerebral infarct
– Hemorrhage
• We present a patient with SCD, chronic left middle cerebral
artery occlusion and Moyamoya disease who develops new
onset of right-sided UPRES.
Case Report
• 12 y.o. male with history of sickle cell disease
• Sudden onset of severe headache, visual
disturbance and hypertension on post
haploidentical stem cell transplant day 20.
• Patient’s clinical and radiologic presentations are
highly compatible with UPRES.
Image Findings
Fig. 1
Fig. 2
Fig. 1 Axial T2-weighted FLAIR (fluid-attenuated inversion recovery) image shows cortical and subcortical
signal hyperintensity in the right temporo-occipital region. Fig. 2 Axial T2-weighted image demonstrates
corresponding T2 signal hyperintensity in the same area.
Image Findings
Fig. 3a
Fig. 3b
Fig. 3 (a an b) Axial diffusion weighted images demonstrate no evidence of restricted diffusion in the right
temporo-occipital region.
Image Findings
Fig. 4a
Fig. 4b
Fig. 4a MR angiography of the anterior circulation reveals occlusion of the left middle cerebral artery.
Fig. 4b MR angiography of the posterior circulation demonstrates no evidence of basilar or posterior
cerebral arterial occlusion.
Image Findings
Fig. 5a
Fig. 5b
Fig. 5 (a and b) Axial T2-weighted images at different levels demonstrate diffuse left cerebral atrophy,
likely related to left middle cerebral artery occlusion and chronic left cerebral infarct.
Image Findings
Fig. 1
Fig. 6
Fig. 1 Axial T2-weighted FLAIR image at presentation shows cortical and subcortical signal hyperintensity
in the right temporo-occipital region. Fig. 6 Two week follow up axial T2-weighted FLAIR image
demonstrates complete resolution of abnormal signal hyperintensity in the right temporo-occipital region.
Summary
• Posterior reversible encephalopathy syndrome
– A reversible neurologic syndrome with a variety of
presenting symptoms and causes.
– Classic PRES shows symmetric bilateral T2 signal
hyperintensity involving the parieto-occipital and
posterior frontal white matter.
– Posterior cerebral circulation predilection.
– Relative lack of arteriole sympathetic innervation in
the posterior circulation.
– Disruption of blood brain barrier and endothelial
damage lead to loss of arteriole autoregulation.
– UPRES only accounts for 2-3% of all patients with
PRES.
Summary
• Sickle cell disease
–
–
–
–
–
Autosomal recessive
Defect in the beta-chain hemoglobin
Microvascular occlusion
Bone infarct and osteomyelitis
Moyamoya disease
• UPRES in a patient with SCD
– Unclear etiology
– Modifying factors may have played a role to give this
unique presentation.
• Left internal carotid artery occlusion
• Chronic left cerebral infarct
Bibliography
•
Beausang-Linder M, Bill A. Cerebral circulation in acute arterial hypertension –
protective effects of sympathetic nervous activity. Acta Physiol Scand. 1981
Feb;111(2):193-9.
•
Landais A, Lemonne N, Etienne-Julan M. Uncommon posterior reversible
encephalopathy syndrome in a sickle-cell patient. J Clin Neurol. 2015 Jul;11(3):287-8.
•
McKinney AM, Short J, Truwit CL, et al. Posterior reversible encephalopathy
syndrome: incidence of atypical regions of involvement and imaging findings. AJR Am
J Roentgenol. 2007 Oct;189(4):904-12.
•
Schwartz RB, Feske SK, Polak JF, et al. Preeclampsia-eclampsia: clinical and
neuroradiographic correlates and insights into the pathogenesis of hypertensive
encephalopathy. Radiology. 2000 Nov;217(2):371-6.