AM McKinney

Christian Lopez Saubidet

Cristina Soler Riera

Darío Scocco

Gabriela Fischer

HP Adams Jr

J Hinchey

JA Hinchey

Leila Haddad

Paulino A Álvarez

RK Garg

VH Lee

WS Bartynski

English

PubMed

Crossref

Atypical imaging findings in a renal transplant patient with reversible posterior leukoencephalopathy syndrome: a case report

Christian Saubidet

Springer - Publisher Connector

BioMed CentralCases Journal
ssOpen AcceCase Report
Atypical imaging findings in a renal transplant patient with 
reversible posterior leukoencephalopathy syndrome: a case report
Cristina Soler Riera*1, Leila Haddad1, Darío Scocco2, Gabriela Fischer3, 
Christian Lopez Saubidet1 and Paulino A Álvarez1
Address: 1Department of Internal Medicine, Centro de Educación Médica e Investigaciones Clínicas (CEMIC), Av Las Heras 2900 (C1425ASS), 
Buenos Aires, Argentina, 2Department of Internal Medicine, Neurology Section, Centro de Educación Médica e Investigaciones Clínicas (CEMIC), 
Av Las Heras 2900 (C1425ASS), Buenos Aires, Argentina and 3Department of Internal Medicine, Nephrology Section, Centro de Educación Médica 
e Investigaciones Clínicas (CEMIC), Av Las Heras 2900 (C1425ASS), Buenos Aires, Argentina
Email: Cristina Soler Riera* - crisoler@telecentro.com.ar; Leila Haddad - hllh1@hotmail.com; Darío Scocco - darioscocco@yahoo.com.ar; 
Gabriela Fischer - gfischersohn@gmail.com; Christian Lopez Saubidet - lopezsaubidet@hotmail.com; 
Paulino A Álvarez - paulinoalvarez@hotmail.com
* Corresponding author    
Abstract
Background: Atypical clinical and imaging findings in Reversible Posterior Leukoencephalopathy
Syndrome are recognized with increasing frequency.
Case report: We report a case of an adult in his 5th decade immunosupressed with
methilprednisolone, tacrolimus and micophenolate who two months after renal transplantation,
multiple infections and an episode of humoral rejection became hypertensive with severe
headaches, visual field abnormalities, seizures, left hemiparesis and hemineglect.
Computed Tomography scan of the brain showed a hypo dense lesion in the left occipital lobe.
Ischemic stroke was diagnosed and aspirin and permissive hypertension were indicated. Twelve
hours later he developed left sided motor seizures and cortical blindness. Magnetic Resonance
Image showed hyper intensity in T2 and FLAIR in both occipital lobes and a small area of cortical
restricted diffusion in Diffuson Weighted Images in the left occipital lobe. With a diagnosis of
Reversible Posterior Leukoencephalopathy Syndrome his blood pressure was controlled with
intravenous labetalol, and two days later the neurologic findings returned to baseline and most
Computed tomography findings resolved.
Conclusion: This case underscores that in the appropriate setting Reversible Posterior
Leukoencephalopathy Syndrome should be suspected and the clinician should not be misled by
atypical clinical or imaging findings. In contrast to other pathologies that resemble Reversible
Posterior Leukoencephalopathy Syndrome, with the right and timely treatment, signs, symptoms
and images can be completely reversible.
Case Presentation
A 44-year-old caucasian man underwent his second kid-
ney transplantation. His induction regimen included thy-
moglobulin, methilprednisolone and rituximab. Because
of increased risk of humoral rejection he underwent 5
plasmapheresis sessions before transplant. He was 1.6 m
tall, and weighted 70 kg.
Published: 30 September 2009
Cases Journal 2009, 2:145 doi:10.1186/1757-1626-2-145
Received: 19 September 2009
Accepted: 30 September 2009
This article is available from: http://www.casesjournal.com/content/2/1/145
© 2009 Soler Riera et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:145 http://www.casesjournal.com/content/2/1/145The maintenance immunosuppressive regimen included
methilprednisolone, tacrolimus and micophenolate.
Delayed graft function occurred and a renal biopsy done
in the first week after transplant showed signs of probable
humoral rejection. Gammaglobulin and plasmapheresis
were started with mild improvement of renal function.
His medical history includes chronic renal failure second-
ary to focal and segmental glomerulonephritis, first renal
transplantation 23 years before the present admission and
on dialysis for the last 10 years, hepatitis C and left eye
blindness of unknown etiology. He had no relevant fam-
ily history and did not smoke or drink alcohol.
During first two months his post-transplant course was
complicated by surgical wound infection, urinary fistula,
nosocomial pneumonia due to Haemophilus influenzae,
and abdominal wall abscess due to Acinetobacter bauma-
nii.
One month after transplant the patient developed severe
left sided headaches and blurred vision. He was hyperten-
sive (150/90 mmHg with a usual blood pressure between
90-60 and 70-50) and a right temporal visual field deficit
was detected. Computed Tomography (CT) showed a left
occipital cortico-subcortical hypodense area. (Figure 1)
Serum creatinine was 3,79 mg/dl, tacrolimus level was 6.8
(normal 5-20), platelets were 137.000/ml. Other labora-
tory test results are shown in table 1.
A Neurology consultant suggested ischemic stroke as the
cause, aspirin was started and permissive hypertension
was allowed.
Twelve hours later he had an episode of left-sided motor
focal seizures followed by left hemiparesis and was admit-
ted to the intensive care unit. He was alert and oriented.
Blood pressure was 165/71, temperature 36.3°C, heart
rate 88, pulse oximetry 100% on room air, respiratory rate
18. The neurological exam showed left arm and left leg
weakness and bilateral amaurosis. A new CT scan showed
bilateral occipital hypodensities and hemorrhagic foci.
(Figure 2) Diphenilhydantoin was started. Brain Magnetic
Resonance Image (MRI) showed bilateral occipital hyper-
intese lesions in Fluid Attenuated Inversion Recovery
(FLAIR) and T2, a small area of cortical left occipital lobe
restricted diffusion on Diffusion Weighted Image (DWI)
and GRE sequence compatible with hemorrhage. (Figure
3, 4, 5, 6) MRI venous and arterial angiography showed
no abnormalities. (Figure 7,8)
With a diagnosis of Reversible Posterior Leukoencepha-
lopathy Syndrome (RPLS) blood pressure was controlled
with intravenous labetalol (2 doses of 10 mg). Neurologic
findings including cortical blindness returned to normal
within 48 hs.
A follow-up CT 17 days later showed decreasing hypoden-
sity in left occipital and parietal lobes with improvement
of the hemorrhagic foci and almost complete resolution
of the right occipital lesions. (Figure 9)
Discussion
RPLS is a clinical radiologic syndrome of heterogeneous
etiologies. In spite of its name the syndrome is not always
reversible, and it is often not confined to either the white
matter or the posterior regions of the brain [1]. Most com-
mon causes are hypertensive encephalopathy, ecclampsia,
treatment with immunosuppressive agents or cytotoxic
drugs and renal failure with hypertension. In solid organ
transplantation the incidence of RPLS is low, between
0.49 and 6% [2].
Since the initial report in 1996 by Hinchey and coworkers
the list of potential offending conditions have been
increasing [3]. Although cyclosporine is the most com-
mon cytotoxic therapy associated with RPLS, it has been
described with other agents including tacrolimus,
sirolimus, cisplatin, interferon, and bevacizumab.
Brain CT without contrast: left occipital cortico-subcortical hypodense areaFigur  1
Brain CT without contrast: left occipital cortico-sub-
cortical hypodense area.Page 2 of 6
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Cases Journal 2009, 2:145 http://www.casesjournal.com/content/2/1/145Among the many described causes of RPLS, hypertension,
tacrolimus, immunoglobulin therapy and renal failure
were present in our patient.
The pathogenesis of RPLS remains unclear, but it appears
to be related to disordered cerebral autoregulation and
endothelial dysfunction. Cytotoxic therapies have direct
toxicity on vascular endothelium [1,3]. RPLS associated
with these therapies may occur in normotensive individu-
als (although blood pressure is usually elevated over base-
line), with nontoxic levels of these drugs, and it can
happen even after several months of exposure to thera-
peutic levels [1,3,4].
The combination of acute hypertension and endothelial
damage results in hydrostatic edema. Hyperperfusion and
edema is mainly seen in the posterior circulation, perhaps
because there is a greater concentration of adrenergic
nerves (thought to be responsible for cerebral autoregula-
tion) around pial and intracerebral vessels in the anterior
circulation than posteriorly.
Common clinical manifestations include headaches,
altered consciousness, visual disturbances, and seizures.
The classic neuroimaging feature is edema involving the
white matter in the posterior portions of the cerebral hem-
Table 1: Laboratory test results
Variable Reference range 28-9-2008 29-9-2008 30-9-2008 16-10-2008
Hematocrit 42-50% 34 34
Hemoglobin 14-17 g/dl 11 11.4
Platelets 150000-300000/ml 131000 131000
Leukocytes 4000 - 10000/ml 4800 6000
Na 136-145 mEq/L 136 138
K 3.5-5.1 mEq/L 5.5 4.7
Creatinine 0.7-1.2 mg/dl 3.79 3.25
BUN 3.73- 23.3 mg/dl 76.44 96.94
Bilirrubin 0.2-1.1 mg/dl 0.7
Total Protein 6.4-8.3 gr/dl 4.4
Quick 70-100% 89%
APTT 30-45 sec 33
Calcium 4.5- 5.5 mEq/L 3.8 4
TGP 10-35 U/L 14
TGO 10-40 U/L 14
Ph 7.37-7.43 7.28 7.37
pCO2 35-45 mmHg 37 25
HCO3- 24-26 milimoles/l 17.7 14
Mg 1.6-2.5 mg/dl 1.5
Tacrolimus 5-20 nanograms/ml 6.8Page 3 of 6
(page number not for citation purposes)
Cases Journal 2009, 2:145 http://www.casesjournal.com/content/2/1/145
Page 4 of 6
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Brain CT without contrast bilateral occipital cortico-subcor-tic l hypodensities and hemorrhagic foFigure 2
Brain CT without contrast bilateral occipital cortico-
subcortical hypodensities and hemorrhagic foci.
Brain MRI FLAIR Bilateral parieto-occipital hyperintese lesionsFigure 3
Brain MRI FLAIR Bilateral parieto-occipital hyperin-
tese lesions.
Brain MRI T2 Bilateral parieto-occipital hyperintese lesionFigure 4
Brain MRI T2 Bilateral parieto-occipital hyperintese 
lesion.
Brain MRI DWI: left occipital cortical restriction in DWIFigure 5
Brain MRI DWI: left occipital cortical restriction in 
DWI.
Cases Journal 2009, 2:145 http://www.casesjournal.com/content/2/1/145ispheres, usually bilaterally in the parieto-occipital
regions. The lesions are seen as white-matter hypodensi-
ties on CT scans and hypointense in T1, hyperintense in
T2 and in FLAIR on MRI studies [5,6]. Atypical distribu-
tions and imaging manifestations such as significant ante-
rior involvement, cortical lesions, recurrent RPLS
episodes, foci of permanent injury, hemorrhage into
lesions, unilaterality, and areas of restricted diffusion are
Brain MRI GRE: left subcortical parieto-occipital hemorragic fociFigure 6
Brain MRI GRE: left subcortical parieto-occipital 
hemorragic foci.
Brain MRI AngiographyFigure 7
Brain MRI Angiography. Normal.
Brain MRI Venous AngiographyFigure 8
Brain MRI Venous Angiography. Normal.
Brain CT without contrastFigure 9
Brain CT without contrast. Decreasing hypodensity in 
left occipital and parietal lobes with improvement of hemor-
rhagic foci and almost complete resolution of right occipital 
lesions.Page 5 of 6
(page number not for citation purposes)
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described with increasing frequency in recent and larger
series of patients [7,8]. Diffusion restriction in RPLS has
been shown to occur in a minority of patients (17% in a
series) and usually is punctuate, small, or patchy and is
surrounded by much larger areas of vasogenic edema
[8,6].
Differential diagnosis include stroke, venous thrombosis,
toxic or metabolic encephalopathy, demyelinating disor-
ders, vasculitis, and encephalitis among others. Particu-
larly in cases with a sudden onset of neurologic
symptoms, as was the case of our patient, the presentation
can mimic bilateral posterior cerebral artery infarctions
("top of the basilar syndrome"). The fact that RPLS usually
spares the calcarine and paramedian occipital lobe helps
to differentiate these two entities [5]. This distinction is
important because in RPLS hypertension should be
treated in order to prevent permanent brain injury and
blood pressure management in ischemic stroke remains
controversial [9]. Furthermore RPLS patients should not
receive antiplatelet treatment.
Conclusion
This reports calls attention to the fact that in the appropri-
ate setting RPLS should be suspected and the clinician
should not be misled by atypical clinical or imaging find-
ings. In contrast to other pathologies that resemble RPLS,
prompt recognition and treatment may prevent perma-
nent damage.
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the written consent is available for
review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
PA, CS, CLS and LH analyzed and interpreted the patient
clinical data and wrote the manuscript.DS and GF inter-
preted data regarding neurological and nephrological dis-
ease respectively. All authors read and approved the final
manuscript.
Acknowledgements
The authors thank Juan Carlos Spina MD, for his contribution in the prep-
aration of this manuscript.
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Atypical imaging findings in a renal transplant patient with reversible posterior leukoencephalopathy syndrome: a case report

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