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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2016  |  Volume : 27  |  Issue : 5  |  Page : 1021-1025
Posterior reversible encephalopathy syndrome in a 5-year-old boy with steroid-dependent nephrotic syndrome

Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Shahbag, Dhaka, Bangladesh

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Date of Web Publication22-Sep-2016


Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and radiological phenomenon is encountered in children compared to adults. In our center, a 5-yearold boy with steroid-dependent nephrotic syndrome (SDNS) presented with headache and blurring of vision during relapse after a long course of immunosuppressive therapy. Evaluation by computed tomography scan of the brain showed that the child had hypodense areas throughout the occipital region of the brain. All signs of PRES, except papilledema, resolved after seven days of supportive treatment evidenced by subsequent radiological evaluation. PRES should be kept in mind in any nephrotic child who is on prolonged immunosuppressive therapy.

How to cite this article:
Rahman M, Qader M, Haque SS, Al Mamun M, Uddin GM. Posterior reversible encephalopathy syndrome in a 5-year-old boy with steroid-dependent nephrotic syndrome. Saudi J Kidney Dis Transpl 2016;27:1021-5

How to cite this URL:
Rahman M, Qader M, Haque SS, Al Mamun M, Uddin GM. Posterior reversible encephalopathy syndrome in a 5-year-old boy with steroid-dependent nephrotic syndrome. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2021 Sep 23];27:1021-5. Available from: https://www.sjkdt.org/text.asp?2016/27/5/1021/190880

   Introduction Top

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological condition characterized by headache, nausea, vomiting, seizures, and visual disturbances with typical radiological features of symmetrical edema mostly involving the white matter in the occipital regions of the brain. [1],[2] Most authors acknowledge that PRES is a disease of adulthood and in pediatric age group, it occurs only rarely. [2] Children who are on prolonged steroid therapy or on calcineurin inhibitor therapy in nephrotic syndrome and chronic kidney disease are in the risk of developing PRES. [3] PRES was first described by Hinchey et al in 1996, where he described 15 cases, among them most of the patients were on maintenance immunosuppression with cyclosporine A (CsA) for aplastic anemia, metastatic melanoma, or after receiving organ transplant. All of them had uncontrolled hypertension. [4] In another study of nine cases in children, authors also observed similar clinical features and imaging pattern, whereas seven patients were on maintenance immunosuppression and another two were on hypertensive crisis. [5] It has been reported that outcome of PRES is satisfactory after withdrawal of the offending drugs or improvement of the etiological factor. [6] Reports of PRES are scarce in the pediatric population. However, there are a few reports of PRES in pediatric age group of patients suffering from CKD or on prolonged calcineurin inhibitor or steroid therapy for nephrotic syndrome. [3],[7]

Here, we are reporting a case of PRES diagnosed in a 5-year-old boy with steroiddependent nephrotic syndrome, who was on prolonged immunosuppression with CsA and prednisolone and developed PRES after six months of discontinuation of CsA.

   Case Report Top

A 5-year old boy having steroid sensitive nephrotic syndrome [Figure 1] admitted in the Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University on July 6, 2013, with the complaints of gradual swelling of the whole body and scanty micturition. On examination, he was found edematous, afebrile, normotensive, and his bedside urine albumin shows (++) proteinuria. As the patient was in a state of relapse, investigations relevant to nephrotic syndromes such as serum albumin, serum total protein, urinary total protein, and serum cholesterol were not done. Although this patient went into spontaneous remission, investigations for infection screening such as complete blood cell, urine R/E and C/S, and X-ray chest were done, which is a usual institutional practice before starting prednisolone therapy. Regarding past medical history about nephrotic syndrome, his initial episode occurred at the age of two years, when he was treated with prednisolone for a period of 12 weeks by a pediatric nephrologist. Subsequently, the patient developed another attack after an interval of six months from recovery of initial episode and achieved remission with treatment of relapse. Previous two attacks occurred within one year of present illness. During 3 rd episode of nephrotic syndrome, he developed steroid dependency and received alkylating agent cyclophosphamide. However, cyclophosphamide was abandoned after two weeks due to development of toxicity, later on, treatment was continued only with prednisolone, without any steroid-sparing drug. As a result, during 4 th episode, the patient was treated with CsA for a period of one year along with corticosteroid in initial three months. Before starting CsA therapy, renal biopsy was done, and minimal change disease was proved by histopathological evaluation. CsA was stopped after one year, due to raised serum creatinine, which became normal after stoppage of CsA. The patient was on remission for six months after stoppage of CsA therapy.
Figure 1: Patient of posterior reversible encephalopathy syndrome.

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During present illness after four days of admission, the patient developed severe headache and blurring of vision without vomiting. His neurological examination revealed nothing consistent with meningitis or dural sinus thrombosis. As per advice of an expert pediatric neurologist, computed tomography (CT) scan of the brain was done which showed hypodense areas throughout the occipital region of the brain [Figure 2]. These typical findings in the brain, consistent with PRES, which is usually noted in the adults in conditions such as prolonged immunosuppressive therapy and renal insufficiency. However, in the pediatric population, it is a rare finding. This time, we continued the treatment of this patient by rest, adequate nutrition, paracetamol, and other general supportive measures. His edema and proteinuria subsided within five days of admission with general measures such as salt and fluid restriction and empirical broad spectrum antibiotic therapy given, suspecting that the patient might have a hidden infection or assuming that antibiotic therapy may bring a relapsing nephrotic child to remission without loading the child with further corticosteroid therapy.
Figure 2: Computed tomography scan shows hypodense area in posterior region.

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Later on, we again consulted the neurologist, neurosurgeon, and ophthalmologist for his headache and visual disturbances. Subsequent evaluation, both by magnetic resonance imaging (MRI) [Figure 3] and magnetic resonance venography (MRV) of the brain [Figure 4], revealed no abnormality of the brain, which is a usual phenomenon in case of PRES. Unfortunately, his eye problems had not improved, and ophthalmological evaluation showed papilledema in both eyes. Ophthalmologist assured that this will resolve in due course of time but needs regular follow-up.
Figure 3: Normal magnetic resonance imaging on follow-up.

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Figure 4: Normal magnetic resonance venography on follow-up.

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Further improvement occurred within next seven days with the patient having no headache and visual disturbances, and he was discharged with necessary advice regarding his illness.

   Discussion Top

PRES was first described by Hinchey et al in 1996, [4] he described 15 cases in a case series, most of the cases were on maintenance immunosuppression for aplastic anemia, metastatic melanoma, or after receiving an organ transplant. He also described four patients with hypertensive encephalopathy either due to systemic lupus erythematosus, idiopathic glomerulonephritis, or hepatorenal syndrome and described three patients with postpartum eclampsia. [4]

PRES is diagnosed by typical clinical presentation and characteristic neuroimaging findings. Radiological diagnosis of PRES can be made via CT scan, but MRI is the most sensitive imaging modality. [8] Several findings in neuroimaging are suggestive of PRES, but most common abnormalities on CT scan and/ or MRI scans are focal regions of vasogenic edema involving the white matter in the posterior cerebral hemisphere, often asymmetrically and most commonly involving the parietooccipital lobes bilaterally, often in a watershed-type distribution. [9] However, different regions may be involved, but findings on CT scan or MRI scan could vary with the presence of hemorrhage or infarcts. [1] The radiological appearance of PRES does not seem to be influenced by the predisposing factors such as nephrotic syndrome, chronic kidney disease, and after receiving an organ transplant. [10]

In cases, PRES related with steroid-resistant nephrotic syndrome or steroid-sensitive nephrotic syndrome is seen in those who had either hypertensive encephalopathy or are on maintenance immunosuppressive therapy (like CsA or FK 506). [12],[13] However, it can also be observed in steroid-sensitive nephrotic syndrome. [14] Hence, if a child with nephrotic syndrome presents with seizures or coma, PRES should be kept in mind as a differential diagnosis and prompt evaluation and management is needed. [11]

Although it is considered as a reversible condition, always may not and can cause secondary complications such as status epilepticus, intracranial hemorrhage, and massive ischemic infarction which can be a cause of substantial morbidity and mortality. [8] In most of the cases, after correction of hypertension or improvement of etiological factors, repeat MRI scan shows improvement or resolution of radiological abnormalities although hemorrhages (seen in approximately 15% of cases) can cause permanent structural damage. [9]

In this case, the presenting features are typical and consistent with that of PRES. At presentation, CT scan of this patient showed hypodensity in the occipital region, which are characteristic findings in PRES. Most of the children, who are diagnosed as PRES are on maintenance immunosuppression or had severe hypertension. However, in our patient, PRES was diagnosed when he was not on immunosuppressive therapy and had normal blood pressure. However, he was previously treated with CsA for one year. On follow-up, MRI scan after 10 days showed complete resolution and MRV shows normal circle of Willis's, but there was persistence of squint on the left eye. Roth et al also observed, there may be mild or severe residual effect. [15]

In this case report, we have also highlighted a potentially important association of PRES with nephrotic syndrome even without the use of immunosuppressant medications.

   Conclusion Top

From this case scenario, conclusion may be drawn that if a child with idiopathic nephrotic syndrome, who was on long continued immunosuppressant, develop headache, visual disturbance, PRES can be one of the most important differential diagnoses.

   References Top

Lee VH, Wijdicks EF, Manno EM, Rabinstein AA. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 2008;65:205-10.  Back to cited text no. 1
Legriel S, Pico F, Azoulay E. Understanding posterior reversible encephalopathy syndrome. In: Vincent L, ed. Annual Update in Intensive Care and Emergency Medicine 2011. Berlin Heidelberg: Springer Science+Business Media LLC; 2011.  Back to cited text no. 2
Ishikura K, Ikeda M, Hamasaki Y, et al. Nephrotic state as a risk factor for developing posterior reversible encephalopathy syndrome in paediatric patients with nephrotic syndrome. Nephrol Dial Transplant 2008;23:2531-6.  Back to cited text no. 3
Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494-500.  Back to cited text no. 4
Incecik F, Hergüner MO, Altunbasak S, Erbey F, Leblebisatan G. Evaluation of nine children with reversible posterior encephalopathy syndrome. Neurol India 2009;57:475-8.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
Covarrubias DJ, Luetmer PH, Campeau NG. Posterior reversible encephalopathy syndrome:Prognostic utility of quantitative diffusionweighted MR images. AJNR Am J Neuroradiol 2002;23:1038-48.  Back to cited text no. 6
Onder AM, Lopez R, Teomete U, et al. Posterior reversible encephalopathy syndrome in the pediatric renal population. Pediatr Nephrol 2007;22:1921-9.  Back to cited text no. 7
Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: Associated clinical and radiologic findings. Mayo Clin Proc 2010;85:427-32.  Back to cited text no. 8
Osborn AG, Blaser SI, Salzman KL, Katzman GL. Diagnostic Imaging: Brain. Salt Lake City, UT: Amirsys; 2004.  Back to cited text no. 9
Mueller-Mang C, Mang T, Pirker A, Klein K, Prchla C, Prayer D. Posterior reversible encephalopathy syndrome: Do predisposing risk factors make a difference in MRI appearance? Neuroradiology 2009;51:373-83.  Back to cited text no. 10
Jorge S, Lopes JA, De Almeida E, Martins Prata M. Posterior reversible encephalopathy syndrome (PRES) and chronic kidney disease. Nefrologia 2007;27:650.  Back to cited text no. 11
Ikeda M, Ito S, Hataya H, Honda M, Anbo K. Reversible posterior leukoencephalopathy in a patient with minimal-change nephrotic syndrome. Am J Kidney Dis 2001;37:E30.  Back to cited text no. 12
Utsumi K, Amemiya S, Iizuka M, Iino Y, Katayama Y. Acute posterior leukoencephalopathy in a patient with nephrotic syndrome. Clin Exp Nephrol 2003;7:63-6.  Back to cited text no. 13
Li Looi J, Christiansen JP. Reversible posterior leukoencephalopathy associated with minimal change nephrotic syndrome. N Z Med J 2006;119:U2257.  Back to cited text no. 14
Roth C, Ferbert A. Posterior reversible encephalopathy syndrome: Long-term follow-up. J Neurol Neurosurg Psychiatry 2010;81:773-7.  Back to cited text no. 15

Correspondence Address:
Md. Habibur Rahman
Department of Pediatric Nephrology, Bangabandhu Sheikh Mujib Medical University, Shahbag, Dhaka 1000
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DOI: 10.4103/1319-2442.190880

PMID: 27752014

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