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 Table of Contents  
CASE REPORT
Year : 2012  |  Volume : 1  |  Issue : 2  |  Page : 107-111

Posterior reversible encephalopathy in a child with acute glomerulonepheritis and malrotated kidney


1 Department of Pediatrics, Faculty of Medicine, Zagazig University, Zagazig, Egypt
2 Department of Radiodiagnosis, Faculty of Medicine, Zagazig University, Zagazig, Egypt

Date of Web Publication13-Sep-2012

Correspondence Address:
Doaa M Youssef
Department of Pediatrics, Faculty of Medicine, Zagazig University, Zagazig 44519
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0521.100967

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  Abstract 

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome which is mostly associated with pre-eclampsia, autoimmune diseases, and cytotoxic medication. Our understanding of this disease is based mainly on case reports which identify causes, pattern, and prognosis, and the reports in children are not frequent. We report a case of acute glomerulonepheritis and PRES, which presented with generalized convulsions and loss of consciousness. On diffusion-weighted magnetic resonance imaging (MRI), the affected areas appeared hyperintense on both diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) maps, which is consistent with unrestricted diffusion in regions of PRES vasogenic edema. MR spectroscopy (MRS) revealed normal metabolite spectrum. Our case was completely reversible after blood pressure control. We conclude that PRES as a possibility should be considered in children with acute glomerulonepheritis with neurological findings, even with status epilepticus presentation, and that we can confirm our suspicion by MRI showing typical bilateral hypodense findings.

Keywords: Children, nephritis, posterior reversible encephalopathy


How to cite this article:
Youssef DM, Fawzy FM. Posterior reversible encephalopathy in a child with acute glomerulonepheritis and malrotated kidney. Saudi J Health Sci 2012;1:107-11

How to cite this URL:
Youssef DM, Fawzy FM. Posterior reversible encephalopathy in a child with acute glomerulonepheritis and malrotated kidney. Saudi J Health Sci [serial online] 2012 [cited 2021 Jan 22];1:107-11. Available from: https://www.saudijhealthsci.org/text.asp?2012/1/2/107/100967


  Introduction Top


The term "reversible posterior leukoencephalopathy" (RPLS) or "posterior reversible encephalopathy syndrome" (PRES) is a clinico-radiological syndrome, manifesting with headache, confusion, seizures, visual disturbances, and radiological findings of bilateral gray and white matter abnormalities suggestive of edema in the posterior regions of cerebral hemispheres. [1]

Described mainly in adults, it has also been reported in children. [2] The imaging pattern is typically seen in patients who develop eclampsia or cyclosporine/FK-506 neurotoxicity after transplantation, but other associations have been reported, including autoimmune disease [such as systemic lupus erythematosus (SLE) or Wegener granulomatosis] and hypertension. The cause of PRES is controversial and unproven. [3]

Early recognition of PRES is important for timely institution of therapy, which typically consists of gradual blood pressure control and withdrawal of potentially offending agents. Although reversible by definition, secondary complications, such as status epilepticus (SE), intracranial hemorrhage, and massive ischemic infarction, can cause substantial morbidity and mortality. Our understanding of PRES is derived primarily from small retrospective studies in which the definition of the syndrome varies; though the range of presentations and clinical associations is still being recognized. [4]


  Case Report Top


An 11-year-old boy presented to Intensive Care Unit, Pediatric Department, Zagazig University, with generalized convulsion which started hours before admission and was difficult to be controlled in primary health care center with two doses of diazepam.

On history taking and examination, we found that the child had hematuria in the last 10 days for which he received nonspecific treatment and it did not improve. On examination, the child had disturbed conscious level with Galsgow coma scale 8\15, temperature was 37.3°C, respiratory rate was 28\min, blood pressure was 190\120 mm Hg, and there was neither edema nor any sign of meningeal irritation.

His blood pressure was controlled with sodium nitroperroside; the convulsions ceased and he regained the conscious level gradually in the following 2 days.

Later on, the child complained of headache and loss of vision; for which fundus examination was done and it was normal.

Computed tomography (CT) revealed bilateral cortical and subcortical hypodense areas at the parieto-occipital region and subtle hypodensity at the fronto-parietal region, with no post-contrast enhancement [Figure 1] and [Figure 2].
Figure 1: Non-contrast axial CT study at the level of the lateral ventricles revealed bilateral parieto-occipital hypodensity with no significant mass effect and subtle hypodense area at the right fronto-parietal region

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Figure 2: Post-contrast CT study at the same level (the lateral ventricles) revealed no significant enhancement of these areas

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Conventional magnetic resonance imaging (cMRI) showed bilateral areas of abnormal signal intensity in cortical and subcortical areas of the parieto-occipital region as well as patchy abnormal signal intensity areas at the fronto-parietal region, and displayed hypointense signal on T1WI [Figure 3] and hyperintense signal on T2WI [Figure 4], more evident on coronal T2WIs with no significant enhancement in post-contrast images using gadolinium-diethylene triamine pentaacetic acid (Gd DTPA) in a dose of 0.1 mmol/kg body weight [Figure 5].
Figure 3: Axial T1WI without Gd DTPA revealed cortical and subcortical abnormal hypointense signal at the parieto-occipital region

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Figure 4: Axial and coronal T2WIs revealed bilateral parieto-occipital hyperintensity and subtle hyperintense signal at the fronto-parietal region

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Figure 5: Axial and coronal T2WIs revealed bilateral parieto-occipital hyperintensity and subtle hyperintense signal at the fronto-parietal region, more evident on the right side on coronal T2WI

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On diffusion-weighted MRI, the affected areas appeared hyperintense on both diffusion-weighted imaging (DWI) [Figure 6],[Figure 7] and apparent diffusion coefficient (ADC) maps [Figure 8], which is consistent with unrestricted diffusion in regions of PRES vasogenic edema.
Figure 6: Coronal T1WI post Gd DTPA revealed no contrast enhancement in the described areas

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Figure 7: DWI and ADC map revealed increased signal intensity of the parieto-occipital region

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Figure 8: DWI and ADC map revealed increased signal intensity of the parieto-occipital region, which is consistent with unrestriction of diffusion in the affected areas by vasogenic edema

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MR spectroscopy (MRS) revealed normal metabolite spectrum regarding the N-acetyl aspartate (NAA), creatine (Cr), and choline (Cho), excluding the presence of neoplastic or infectious insult; however, lactate (Lac) peak was reported to be slightly elevated above the baseline at 1.3 ppm (part per million), suggesting the element of ischemia as seen in the PRES with vasoconstriction [Figure 9] and [Figure 10].
Figure 9: Multi-voxel MRS revealed no significant changes in the normally presented metabolite on the MR spectrum; however, mild lactate peak was observed at 1.3 ppm, which carried an element of ischemia in our case

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Figure 10: Multi-voxel MRS revealed no significant changes in the normally presented metabolite on the MR spectrum; however, mild lactate peak was observed at 1.3 ppm, which carried an element of ischemia in our case

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Urine analysis showed turbidity, albumin ++, RBCs 30-35\high-power field (HPF), WBCs 35-40\HPF, and 24-h urinary protein 972 mg\24 h. Urine culture showed no pathological growth, serum albumin 3.1 g\dl, serum urea 108 mg\dl, serum creatinine 1.29 mg\dl, C-reactive protein (CRP) negative, Na 145 mEq\L, K 4.3 mEq\L, Ca 8 mg\dl, Mg 1.8 mg\ dl, Hb 8.4 gm%, platelet 357,000 concentration counts/ μL, WBCs 7.2000, erythrocyte sedimentation rate (ESR) 1 st h 17, lactate dehydrogenase (LDH) 547, antinuclear antibody (ANA) negative, low C3 0.5 g/L (0.9-1.8), and normal C4 0.2 g/L (0.1-0.4). Antistreptolysin O titer (ASOT) was positive 1\400.

Both cholesterol (173 mg\dl) and Triglycerides (TGSs) (137 mg\dl) were normal.

Renal biopsy done after 4 days of admission revealed resolving diffuse proliferative exudative glomerulonephritis.

Ultrasonography (US) revealed normal site, size, parenchymal thickness of both kidneys and good cortico-medullary differentiation with element of malrotation of the left pelvi-calyceal system as the renal pelvis was facing laterally, but with no solid or cystic renal masses, stones, or hydronephrotic changes. Intravenous urography (IVU)confirmed the malrotation of the left kidney as the renal pelvis was dilated and directed laterally and the calyces were directed medially relative to the right kidney. However, it displayed normal excretory function and normal contrast steaming through the pelvi-calyceal system [Figure 11].
Figure 11: IVU revealed malrotation of the left kidney evidenced by the maldirection of the pelvi-calyceal system as the renal pelvis was directed laterally and the calices were facing medially

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The patient was discharged after 7 days of admission, on oral diuretics (frusamide) single dose to control and maintain the blood pressure, with follow-up for the next month with the antihypertensive withdrawn. After 7 weeks, the C3 measured again showed normal level of 1 g/L (0.9-1.8) and C4 showed 0.2 g/L (0.1-0.4), and MRI showed gradual regression of the abnormal signal intensity areas in the subsequent MRI follow-up [Figure 12], which was parallel to the gradual improvement in the patient's general condition.
Figure 12: Follow-up MRI shows regression of the abnormal signal intensity areas

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  Discussion Top


We diagnosed our case as a case of PRES with acute glomerulonepheritis as he fulfilled the criteria of this clinico-radiological syndrome. The case presented to us with convulsions with hypertension and a history of hematuria. On controlling the convulsion with anticonvulsant and antihypertensive, he complained of impaired vision with headache, which is the major clinical presentation of PRES. As regards the radiological findings, he showed bilateral cortical and subcortical hypodense areas in the parieto-occipital region on CT, with subtle hypodensity at the fronto-parietal region, which correlated with cMRI that revealed bilateral areas of abnormal signal intensity at the parieto-occipital region as well as fronto-parietal patchy areas of abnormal signal intensity, and displayed hypointense signal on T1WI and increased signal intensity on T2WI with no significant contrast enhancement. On DWI and ADC map, these areas displayed hyperintense signal (unrestricted diffusion), while MRS revealed no significant changes in the normal metabolite peaks apart from the slight elevation of Lac peak, which is an indication of an ischemic element. Follow-up MRI revealed gradual improvement of the abnormal signal intensity areas detected on the conventional and functional imaging, which was parallel to the improvement in the general condition of the patient.

Our case presented with status epilepticus and this clinical presentation of PRES is not uncommon. [4] Wirrell [5] and his colleagues reported four previously well children who presented acutely with altered mentation, seizures, and visual disturbances, and were diagnosed with PRES. Of them, only one child had a history of gross hematuria prior to the seizure. All four were discovered to be hypertensive only after the onset of their neurological symptoms, and were subsequently diagnosed to be glomerulonephritis.

Our patient had mainly a posterior MRI finding and this matches the commonest brain MRI findings reviewed independently by two neuroradiologists, which revealed the parieto-occipital head region to be the region most consistently involved, followed by the frontal lobe, temporal lobe, and cerebellum. [4] MRI abnormalities in the form of T2WI cortical and subcortical hyperintense signal at the parieto-occipital region and to a lesser extent at the fronto-parietal region match the neuroimaging of PRES that is typically associated with high signal intensity on T2-weighted images, predominantly in the posterior regions, which is caused by subcortical white matter vasogenic edema. [6] The hyperintensity detected on DWI and ADC map establishes and consistently demonstrates that the areas of abnormality represent vasogenic edema. [7] The slight elevation of Lac peak reported in our patient suggested the presence of an ischemic element subsequent to vasoconstriction matched the MR spectrum finding of Lac peak that has been reported in PRES when accompanied with vasoconstriction, a contribution from ischemia has been suggested. [8]


  Conclusion Top


We conclude that PRES should be considered in children with acute glomerulonepheritis with neurological findings and that we can confirm our suspicion by MRI showing typical bilateral hypodense findings.


  Acknowledgments Top


The authors acknowledge professors at the Pediatrics and Radiodiagnosis departments in Zagazig University Hospital for helping the authors in editing and revision of this case.

 
  References Top

1.Kwon S, Koo J, Lee S. Clinical spectrum of reversible posterior leucoencephalopathy syndrome. Pediatr Neurol 2001;24:361-4.  Back to cited text no. 1
[PUBMED]    
2.Gupta SH, Goyl VK, Talukdar B. Reversible posterior leucoencephalopathy syndrome in post streptococcal glomerulonephritis. Indian Pediatr 2010;47:274-6.  Back to cited text no. 2
    
3.Covarrubias DJ, Leutmer PH, Campeau NG. Posterior reversible encephalopathy syndrome: Prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol 2002;23:1038-48.  Back to cited text no. 3
    
4.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. 4
[PUBMED]    
5.Wirrell EC, Hamiwka LD, Hamiwka LA, Grisaru S, Wei X. Acute glomerulonephritis presenting with PRES: A report of 4 cases. Can J Neurol Sci 2007;34:316-21.  Back to cited text no. 5
[PUBMED]    
6.Ahn KJ, You WJ, Jeong SL, Lee JW, Kim BS, Lee JH, et al. Atypical manifestations of reversible posterior leukoencephalopathy syndrome: Findings on diffusion imaging and ADC mapping Neuroradiology 2004;46:978-83.  Back to cited text no. 6
    
7.Mukherjee P, McKinstry RC. Reversible posterior leukoencephalopathy syndrome: Evaluation with diffusion-tensor MR imaging. Radiology 2001;219:756-65.   Back to cited text no. 7
[PUBMED]    
8.Kwon S, Koo J, Lee S. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Pediatr Neurol 2001;24:361-64.  Back to cited text no. 8
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12]



 

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Introduction
Case Report
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