Letter to the Editor

Iran J Public Health, Vol. 49, No.7, Jul 2020, pp.1390-1391

A Case-Study of a Child with Reversible Posterior
Leukoencephalopathy Syndrome (RPLS) Associated with SevereBurns throughout the BodyKoji WAKE 1, *George IMATAKA 2, Toshihiko OHNISHI 1, Jin KIKUCHI 1, Eisei
HOSHIYAMA 1, Kazuyuki ONO 1, Shigemi YOSHIHARA 2
1. Department of Emergency and Critical Care Medicine, Dokkyo Medical University, Tochigi, Japan
2. Department of Pediatrics, Dokkyo Medical University, Tochigi, Japan*Corresponding Author: Email: geo@dokkyomed.ac.jp(Received 24 Sep 2019; accepted 16 Oct 2019)

Dear Editor-in-Chief
Reversible posterior leukoencephalopathy syndrome (RPLS) was first reported by Hinchey et alin 1996 (1). In recent years, the same pathologicalcondition has been called posterior reversible encephalopathy syndrome (PRES) (2). RPLS/PRES
is characterized by the development of acute encephalopathy associated with hypertension due tovarious factors. Clinical symptoms include headache, vomiting, disturbance of consciousness,convulsions, cortical- and half-blindness, as well
as visual impairment such as visual space neglect.
Results obtained from brain magnetic resonanceimaging (MRI)/ computed tomography (CT)scans of RPLS/PRES showed abnormal lesions
in the occipital and parietal lobe areas (2). These
symptoms are often after treatment of the underlying diseases (2). Here in this paper, we report
the case of a child who developed RPLS/PRES
during the treatment for severe burns throughout
his body.
A healthy 13-year-old boy who suffered severe
body burns from a fire at his home, was transferred to the emergency center of our hospital
unit. According to the skin color tone, depth, andrule of nines, his 45% of his burns classified as
deep grade Ⅲ, and 30% were grade II deep skinburns. The calculated burn index was 60. Becauseof a burn in his airway, we immediately started
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ventilator treatment under general anesthesia.
After 18 days of treatment in ICU, the patientadvanced from artificial to spontaneous respiration. In the meantime, we used a sufficientamount of sedatives and analgesics, and performed daily skin disinfection. He also received
several skin graft transplants during this period.
On day 50, the patient underwent a fifth skingraft under general anesthesia. On the 51st day, hecomplained about visual impairment, indicating aform of blindness. He visited an ophthalmologist,
and there were no abnormalities in the light reflection, fundus, or anterior segment of the eyes.
His consciousness level was E4V5M6 according
to GCS scoring. His body temperature was 37.7℃, with a respiratory rate of 22 times/min. His
heart rate was 86 times/min and had high bloodpressure (150/89 mmHg). We determined that
there was a psychiatric element and watched thecourse with sedatives. Patient suffered from convulsions on day 53, and a blood test showed anincrease in sodium (139 mEq/L) and potassium(2.3 mEq/L), along with an overall increase inthe inflammatory response. Head CT was thenperformed, and a low-density area with regions ofhigh-density was found inside the bilateral occipital lobe (Fig. 1A and B). On day 54, MRI confirmed high intensity on diffusion weighted imAvailable at:

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Wake et al.: A Case-Study of a Child with Reversible Posterior Leukoencephalopathy …

ages (DWI) (Fig. 1C and D) and ADC-mapping(Fig. 1E and F) on both occipital sides.

Fig. 1: MRI findings on 54th day from the onset

We diagnosed reversible posterior leukoencephalopathy syndrome (PRES) based on his clinicalcourse. The convulsions were well controlledwith propofol and midazolam. Hypertension wasrelieved with administration of nicardipine. On
day 61, MRI revealed abnormal intensity in both
occipital regions with T2 weighted image andFLAIR (Fig. 2A and B). His visual impairment
was recovered completely on day 64. By the day75, a brain MRI was performed and results
showed a decline in the intensity of abnormalities(Fig. 2C and D). Abnormal intensity improved
further on 113th day (Fig. 2E and F). The patient
was discharged without motor paralysis and sequelae.

There are two major hypotheses for the development of RPLS/PRES. One is the “breakthrough” hypothesis, in which angiogenic edema
is caused by the breakdown of the blood brain
barrier associated with an increase in blood pressure. Alternatively, the “Vasospasm” hypothesis
proposes that RPLS/PRES is caused by cerebral
vasospasm. Recent studies suggested that thepathogenesis of RPLS/PRES was associated withthese two mechanisms. In addition, brain MRI
scans suggest that RPLS/PRES is a pathologicalcondition in which vasogenic edema and/or cytotoxic edema is reversible. In our case, we considered that hypertensive encephalopathy wascaused by several factors, including an increase inblood pressure, blood vessel dilation and angioedema caused by fluctuations in hemodynamics
during burn treatment, as well as stress during theperioperative period of skin grafting. As hypertension is known to be a risk factor forRPLS/PRES (3). We believe that, based on theclinical case presented here, an increase in bloodpressure associated with severe burns should beconsidered as an important factor that has thepotential to trigger RPLS/PRES.

Conflict of Interest
The authors declare that there is no conflict ofinterest.

References
1. Hinchey J, Chaves C, Appignani B, et al (1996).
A Reversible Posterior Leukoencephalopathy
Syndrome. N Engl J Med, 334(8): 494-500.
2. Tetsuka S, Ogawa T (2019). Posterior reversible
encephalopathy syndrome: A review will emphasis on neuroimaging characteristics. J Neurol Sci, 15: 404-472.
3. Liman TG, Siebert E, Endres M (2019). Posterior reversible encephalopathy syndrome. Curr
Opin Neurol, 32(1): 25-35.
Fig. 2: Serial MRI studies on day 61(A/B), 75(C/D)
and 113(E/F)

Available at:

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