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Posterior reversible encephalopathy syndrome in children with kidney disease

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Abstract

Posterior reversible encephalopathy syndrome (PRES) has been described as a neurological condition observed in a variety of clinical settings and is characterized by focal neurological deficits, seizures, headaches, altered mental status, and visual impairment, associated with transient typical lesions on neuroimaging, predominantly in the posterior part of the brain. The most common risk factors for PRES are hypertension, renal diseases, and the use of calcineurin inhibitors. The incidence of PRES in children with renal disorders varies between 4 and 9%, according to different reports. Vasogenic cerebral edema is considered the major pathophysiological mechanism of PRES. There are two main theories regarding the genesis of this edema: (1) hyperperfusion, due to autoregulatory failure of the cerebral vasculature, and (2) hypoperfusion, due to vasoconstriction of the cerebral arteries. In addition, PRES might also be the result of a systemic inflammatory state causing endothelial dysfunction. The management of PRES includes BP control, treatment of seizures, and removal of or reduction in calcineurin inhibitors. Intravenous administration of antihypertensive therapy is preferred, and various drugs have been used in this regard, including nicardipine, labetalol, sodium nitroprusside, and hydralazine. The prognosis of PRES is usually benign, except for rare cases with intracranial hemorrhage.

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References

  1. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, Pessin MS, Lamy C, Mas JL, Caplan LR (1996) A reversible posterior leukoencephalopathy syndrome. N Engl J Med 334:494–500

    Article  CAS  PubMed  Google Scholar 

  2. Yamada A, Ueda N (2012) Age and gender may affect posterior reversible encephalopathy syndrome in renal disease. Pediatr Nephrol 27(2):277–283

    Article  PubMed  Google Scholar 

  3. Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Shishido S, Asanuma H, Nishimura G, Hiramoto R, Honda M (2006) Posterior reversible encephalopathy syndrome in children: its high prevalence and more extensive imaging findings. Am J Kidney Dis 48:231–238

    Article  PubMed  Google Scholar 

  4. Ishikura K, Hamasaki Y, Sakai T, Hataya H, Mak RH, Honda M (2012) Posterior reversible encephalopathy syndrome in children with kidney diseases. Pediatr Nephrol 27(3):375–384

    Article  PubMed  Google Scholar 

  5. Onder AM, Lopez R, Teomete U, Francoeur D, Bhatia R, Knowbi O, Hizaji R, Chandar J, Abitbol C, Zilleruelo G (2007) Posterior reversible encephalopathy syndrome in the pediatric renal population. Pediatr Nephrol 22:1921–1929

    Article  PubMed  Google Scholar 

  6. Ikeda M, Ito S, Hataya H, Honda M, Anbo K (2001) Reversible posterior leukoencephalopathy in a patient with minimal-change nephrotic syndrome. Am J Kidney Dis 37:E30

    Article  CAS  PubMed  Google Scholar 

  7. Stott VL, Hurrell MA, Anderson TJ (2005) Reversible posterior leukoencephalopathy syndrome: a misnomer reviewed. Intern Med J 35:83–90

    Article  CAS  PubMed  Google Scholar 

  8. Prasad N, Gulati S, Gupta RK, Kumar R, Sharma K, Sharma RK (2003) Is reversible posterior leukoencephalopathy with severe hypertension completely reversible in all patients? Pediatr Nephrol 18:1161–1166

    Article  PubMed  Google Scholar 

  9. Casey SO, Sampaio RC, Michel E, Truwit CL (2000) Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 21:1199–1206

    CAS  PubMed  Google Scholar 

  10. Singh N, Bonham A, Fukui M (2000) Immuno suppressive associated leukoencephalopathy in organ transplant recipients. Transplantation 69:467–472

    Article  CAS  PubMed  Google Scholar 

  11. Schwartz RB (2002) Hyperperfusion encephalopathies: hypertensive encephalopathy and related conditions. Neurologist 8:22–34

    Article  PubMed  Google Scholar 

  12. Dillon WP, Rowley H (1998) The reversible posterior cerebral edema syndrome. AJNR Am J Neuroradiol 19:591

    CAS  PubMed  Google Scholar 

  13. Pavlakis SG, Frank Y, Chusid R (1999) Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathy: three names for an old syndrome. J Child Neurol 14:277–281

    Article  CAS  PubMed  Google Scholar 

  14. Chandramohan V, Nagarajan VP, Sathyamoorthi MS et al (2012) Posterior reversible encephalopathy syndrome in a child with autoimmune lymphoproliferative syndrome: case report and review of literature. J Pediatr Neurosci 7(3):221–224

    Article  PubMed  PubMed Central  Google Scholar 

  15. Arzanian MT, Shamsian BS, Karimzadeh P, Kajiyazdi M, Malek F, Hammoud M (2014) Posterior reversible encephalopathy syndrome in pediatric hematologic-oncologic disease: literature review and case presentation. Iran J Child Neurol 8(2):1–10

    PubMed  PubMed Central  Google Scholar 

  16. Gafton B, Porumb V, Ungurianu S, Marinca MV, Cocea C, Croitoru A, Balan G, Miron N, Eliade Ciuleanu T, Miron L (2014) Hepatocellular carcinoma: insights in the biological treatment beyond sorafenib. J BUON 19(4):858–866

    PubMed  Google Scholar 

  17. Endo A, Fuchigami T, Hasegawa M, Hashimoto K, Fujita Y, Inamo Y, Mugishima H (2012) Posterior reversible encephalopathy syndrome in childhood: report of four cases and review of the literature. Pediatr Emerg Care 28(2):153–157

    Article  PubMed  Google Scholar 

  18. El-Naggari MA, Al-Nabhani D, El-Nour I, El-Manzalawy A, Abdelmogheth A-AA (2015) Posterior reversible encephalopathy syndrome in two Omani children with underlying renal diseases. Sult Qaboos Univ Med J 15(3):424–428

    Article  Google Scholar 

  19. Saeed B, Abou-Zor N, Amer Z, Kanani I, Hilal M (2008) Cyclosporin-A induced posterior reversible encephalopathy syndrome. Saudi J Kidney Dis Transpl 19(3):439–442

    PubMed  Google Scholar 

  20. Gera DN, Patil SB, Iyer A, Kute VB, Gandhi S, Kumar D, Trivedi HL (2014) Posterior reversible encephalopathy syndrome in children with kidney disease. Indian J Nephrol 24(1):28–34

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Tenta M, Uchida HA, Nunoue T, Umebayashi R, Okuyama Y, Kitagawa M, Maeshima Y, Sugiyama H, Wada J (2015) Successful treatment by mycophenolate mofetil in a patient with focal segmental glomerulosclerosis associated with posterior reversible encephalopathy syndrome. CEN Case Rep 4(2):190–195

    Article  PubMed  Google Scholar 

  22. Sakai N, Kawasaki Y, Imaizumi T, Kanno S, Go H, Mitomo M, Ushijima Y, Suyama K, Ito M, Hashimoto K, Hosoya M (2010) Two patients with focal segmental glomerulosclerosis complicated by cyclosporine-induced reversible posterior leukoencephalopathy syndrome. Clin Nephrol 73:482–486

    Article  CAS  PubMed  Google Scholar 

  23. Nakahara C, Hasegawa N, Izumi I, Kanemoto K, Iwasaki N (2005) The use of cyclosporine in a boy with a prior episode of posterior encephalopathy. Pediatr Nephrol 20:657–661

    Article  PubMed  Google Scholar 

  24. Soylu A, Kavukcu S, Turkmen M, Akbas Y (2001) Posterior leukoencephalopathy syndrome in poststreptococcal acute glomerulonephritis. Pediatr Nephrol 16:601–603

    Article  CAS  PubMed  Google Scholar 

  25. Ohta T, Sakano T, Shiotsu M, Furue T, Ohtani H, Kinoshita Y, Mizoue T, Kiya K, Tanaka I (2004) Reversible posterior leukoencephalopathy in a patient with Wegener granulomatosis. Pediatr Nephrol 19:442–444

    Article  PubMed  Google Scholar 

  26. Bartynski WS (2008) Posterior reversible encephalopathy syndrome. I. Fundamental imaging and clinical features. AJNR Am J Neuroradiol 29:1036–1042

    Article  CAS  PubMed  Google Scholar 

  27. Roth C, Ferbert A (2011) The posterior reversible encephalopathy syndrome: What’s certain, what’s new? Pract Neurol 11:136–144

    Article  CAS  PubMed  Google Scholar 

  28. Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA (2010) Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc 85:427–432

    Article  PubMed  PubMed Central  Google Scholar 

  29. McKinney AM, Short J, Truwit CL, McKinney ZJ, Kozak OS, SantaCruz KS et al (2007) Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings. AJR Am J Roentgenol 189:904–912

    Article  PubMed  Google Scholar 

  30. Hobson EV, Craven I, Blank SC (2012) Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness. Peritoneal Dial Int J Int Soc Peritoneal Dial 32(6):590–594

    Article  Google Scholar 

  31. Bechstein WO (2000) Neurotoxicity of calcineurin inhibitors: impact and clinical management. Transpl Int 13:313–326

    Article  CAS  PubMed  Google Scholar 

  32. Bartynski WS (2008) Posterior reversible encephalopathy syndrome. II. Controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 29:1043–1049

    Article  CAS  PubMed  Google Scholar 

  33. De Laat P, te Winkel ML, Devos AS, Catsman-Berrevoets CE, Pieters R, van den Heuvel-Eibrink MM (2011) Posterior reversible encephalopathy syndrome in childhood cancer. Ann Oncol 22:472–478

    Article  PubMed  Google Scholar 

  34. Magnasco A, Rossi A, Catarsi P, Gusmano R, Ginevri F, Perfumo F, Ghiggeri GM (2008) Cyclosporin and organ specific toxicity: clinical aspects, pharmacogenetics and perspectives. Curr Clin Pharmacol 3:166–173

    Article  CAS  PubMed  Google Scholar 

  35. Mueller-Mang C, Mang T, Pirker A, Klein K, Prchla C, Prayer D (2009) Posterior reversible encephalopathy syndrome: Do predisposing factors make a difference in MRI appearance? Neuroradiology 51:373–383

    Article  PubMed  Google Scholar 

  36. Jones BV, Egelhoff JC, Patterson RJ (1997) Hypertensive encephalopathy in children. AJNR Am J Neuroradiol 18:101–106

    CAS  PubMed  Google Scholar 

  37. de Oliveira RA, Fechine LM, Neto FC, Nicodemus JM, Silva GB Jr, Silva LS (2008) Posterior reversible encephalopathy syndrome (PRES) induced by cyclosporine use in a patient with collapsing focal glomeruloesclerosis. Int Urol Nephrol 40(4):1095–1098

    Article  PubMed  Google Scholar 

  38. Akutsu N, Iwashita C, Maruyama M, Ootsuki K, Ito T, Saigo K, Kenmochi T (2008) Two cases of calcineurin inhibitor-associated reversible posterior leukoencephalopathy syndrome in renal transplant recipients. Transpl Proc 40:2416–2418

    Article  CAS  Google Scholar 

  39. Ishikura K, Ikeda M, Hamasaki Y, Hataya H, Nishimura G, Hiramoto R, Honda M (2008) Nephrotic state as a risk factor for developing posterior reversible encephalopathy syndrome in paediatric patients with nephrotic syndrome. Nephrol Dial Transpl 23(8):2531–2536

    Article  Google Scholar 

  40. Mueller AR, Platz KP, Bechstein WO et al (1994) Neurotoxicity after orthotopic liver transplantation. A comparison between cyclosporine and FK506. Transplantation 58:155–170

    Article  CAS  PubMed  Google Scholar 

  41. Incecik F, Herguner MO, Altunbasak S, Erbey F, Leblebisatan G (2009) Evaluation of nine children with reversible posterior encephalopathy syndrome. Neurol India 57:475–477

    Article  PubMed  Google Scholar 

  42. Bai JPF, Lesko LJ, Burckart GJ (2010) Understanding the genetic basis for adverse effects: the calcineurin inhibitors. Pharmacotherapy 30:195–209

    Article  CAS  PubMed  Google Scholar 

  43. Qin W, Tan CY, Huang X, Huang Z, Tao Y, Fu P (2011) Rapamycin-induced posterior reversible encephalopathy in a kidney transplantation patient. Int Urol Nephrol 43(3):913–916

    Article  PubMed  Google Scholar 

  44. Becquet O, Pasche J, Gatti H, Chenel C, Abely M, Morville P, Pietrement C (2010) Acute post-streptococcal glomerulonephritis in children of French Polynesia: a 3-year retrospective study. Pediatr Nephrol 25:275–280

    Article  PubMed  Google Scholar 

  45. Gomez-Lado C, Martinon-Torres F, Alvarez-Moreno A, Eiris- Punal J, Carreira-Sande N, Rodriguez-Nunez A, Castro-Gago M (2007) Reversible posterior leukoencephalopathy syndrome: an infrequent complication in the course of haemolytic-uremic syndrome. Rev Neurol 44:475–478

    CAS  PubMed  Google Scholar 

  46. Punaro M, Abou-Jaoude P, Cimaz R, Ranchin B (2007) Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus. Lupus 16:576–579

    Article  CAS  PubMed  Google Scholar 

  47. Zhang YX, Liu JR, Ding MP, Huang J, Zhang M, Jansen O, Deuschl G, Eschenfelder CC (2008) Reversible posterior encephalopathy syndrome in systemic lupus erythematosus and lupus nephritis. Intern Med 47:867–875

    Article  PubMed  Google Scholar 

  48. Ozcakar ZB, Ekim M, Fitoz S, Teber S, Hizel S, Acar B, Yuksel S, Yalcinkaya F (2004) Hypertension induced reversible posterior leukoencephalopathy syndrome: a report of two cases. Eur J Pediatr 163:728–730

    Article  PubMed  Google Scholar 

  49. Benoist G, Dossier C, Elmaleh M, Dauger S (2013) Posterior reversible encephalopathy syndrome revealing renal artery stenosis in a child. BMJ Case Rep 23:2013

    Google Scholar 

  50. Sharma S, Gupta R, Sehgal R, Aggarwal KC (2014) Atypical presentation of posterior reversible encephalopathy: in a child with bilateral grade IV vesicoureteric reflux. J Trop Pediatr 60:331–333

    Article  PubMed  Google Scholar 

  51. Girişgen İ, Tosun A, Sönmez F, Özsunar Y (2010) Recurrent and atypical posterior reversible encephalopathy syndrome in a child with peritoneal dialysis. Turk J Pediatr 52:416–419

    PubMed  Google Scholar 

  52. Awan AQ, Lewis MA, Postlethwaite RJ, Webb NJA (1999) Seizures following renal transplantation in childhood. Pediatr Nephrol 13:275–277

    Article  CAS  PubMed  Google Scholar 

  53. Zheng W (2001) Neurotoxicology of the brain barrier system: new implications. Clin Toxicol 39:711–719

    CAS  Google Scholar 

  54. Tuor UI, Grewal D (1994) Autoregulation of cerebral blood flow: influence of local brain development and postnatal age. Am J Physiol 267:H2220–H2228

    CAS  PubMed  Google Scholar 

  55. Kwon S, Koo J, Lee S (2001) Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Pediatr Neurol 24:361–364

    Article  CAS  PubMed  Google Scholar 

  56. Lee VH, Wijdicks EF, Manno EM, Rabinstein AA (2008) Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 65:205–210

    PubMed  Google Scholar 

  57. Schmithorst VJ, Holland SK, Dardzinski BJ (2008) Developmental differences in white matter architecture between boys and girls. Hum Brain Mapp 29:696–710

    Article  PubMed  PubMed Central  Google Scholar 

  58. Hughes RL (1990) Cyclosporine-related central nervous system toxicity in cardiac transplantation. N Engl J Med 323:420–421

    Article  CAS  PubMed  Google Scholar 

  59. Schwartz RB, Bravo SM, Klufas RA, Hsu L, Barnes PD, Robson CD, Antin JH (1995) Cyclosporine neurotoxicity and its relationship to hypertensive encephalopathy: CT and MR findings in 16 cases. AJR Am J Roentgenol 165:627–631

    Article  CAS  PubMed  Google Scholar 

  60. Ong B, Bergin P, Heffernan T, Stuckey S (2009) Transient seizure-related MRI abnormalities. J Neuroimaging 19:301–310

    Article  PubMed  Google Scholar 

  61. Peter P, George A (2012) Posterior reversible encephalopathy syndrome and the pediatric population. J Pediatr Neurosci 7(2):136–138

    Article  PubMed  PubMed Central  Google Scholar 

  62. Covarrubias DJ, Luetmer PH, Campeau NG (2002) Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol 23:1038–1048

    PubMed  Google Scholar 

  63. Pande AR, Ando K, Ishikura R, Nagami Y, Takada Y, Wada A, Watanabe Y, Miki Y, Uchino A, Nakao N (2006) Clinicoradiological factors influencing the reversibility of posterior reversible encephalopathy syndrome: a multicenter study. Radiat Med 24:659–668

    Article  PubMed  Google Scholar 

  64. Ahn KJ, You WJ, Jeong SL, Lee JW, Kim BS, Lee JH et al (2004) Atypical manifestations of reversible posterior leukoencephalopathy syndrome: findings on diffusion imaging and ADC mapping. Neuroradiology 46:978–983

    Article  CAS  PubMed  Google Scholar 

  65. Bartynski WS, Boardman JF (2007) Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 28:1320–1327

    Article  CAS  PubMed  Google Scholar 

  66. Servillo G, Bifulco F, De Robertis E, Piazza O, Striano P, Tortora F, Striano S, Tufano R (2007) Posterior reversible encephalopathy syndrome in intensive care medicine. Intensive Care Med 33:230–236

    Article  PubMed  Google Scholar 

  67. Eichler FS, Wang P, Wityk RJ (2002) Diffuse metabolic abnormalities in reversible posterior leukoencephalopathy syndrome. AJNR Am J Neuroradiol 23:833–837

    PubMed  Google Scholar 

  68. Bartynski WS, Boardman JF (2008) Catheter angiography, MR angiography, and MR perfusion in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 29:447–455

    Article  CAS  PubMed  Google Scholar 

  69. Wright RR, Mathews KD (1996) Hypertensive encephalopathy in childhood. J Child Neurol 11:193–196

    Article  CAS  PubMed  Google Scholar 

  70. Paues J, Vrethem M (2010) Fatal progressive multifocal leukoencephalopathy in a patient with non-Hodgkin lymphoma treated with rituximab. J Clin Virol 48:291–293

    Article  PubMed  Google Scholar 

  71. Kumar D (2010) Emerging viruses in transplantation. Curr Opin Infect Dis 23:374–378

    PubMed  Google Scholar 

  72. Ko MW, Liu GT (2010) Pediatric idiopathic intracranial hypertension (pseudotumor cerebri). Horm Res Paediatr 74:381–389

    Article  CAS  PubMed  Google Scholar 

  73. Lamy C, Oppenheim C, Meder JF, Mas JL (2004) Neuroimaging in posterior reversible encephalopathy syndrome. J Neuroimaging 14:89–96

    Article  CAS  PubMed  Google Scholar 

  74. llner A (2003) In: Blaser SI (ed) Pocket radiologist PedsNeuro. Salt Lake City, UT, medical reference. PRES, New York, pp 220–222

    Google Scholar 

  75. Parvex P, Pinsk M, Bell LE, O’Gorman AM, Patenaude YG, Gupta IR (2001) Reversible encephalopathy associated with tacrolimus in pediatric renal transplants. Pediatr Nephrol 16:537–542

    Article  CAS  PubMed  Google Scholar 

  76. Gavrilovici C, Oprea L (2013) Clinical ethics, research ethics and community ethics-the moral triad of nowadays society. Revista Romana De Bioetica 11(3):3–5

    Google Scholar 

  77. Gavrilovici C, Goldsmith DJ, Reid C, Gubeth-Tatomir P, Covic A (2004) What is the role of ambulatory BP monitoring in pediatric nephrology? J Nephrol 17(5):642–652

    PubMed  Google Scholar 

  78. Abe K (2004) Reversible posterior leukoencephalopathy syndrome. Intern Med 43:900–901

    Article  PubMed  Google Scholar 

  79. Nishida M, Sato H, Kobayashi N, Morimoto M, Hamaoka K (2009) Wernicke’s encephalopathy in a patient with nephrotic syndrome. Eur J Pediatr 168:731–734

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. “Sf. Maria” Children’s Hospital, Pediatric Department, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania

    Cristina Gavrilovici, Ingrith Miron & Magdalena Stârcea

  2. Nephrology Department, “Dr C. I. Parhon” Hospital, University of Medicine and Pharmacy “Gr. T. Popa”, Iasi, Romania

    Luminiţa Voroneanu & Silvia Bădărau

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  1. Cristina Gavrilovici
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  2. Ingrith Miron
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Correspondence to Cristina Gavrilovici.

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Gavrilovici, C., Miron, I., Voroneanu, L. et al. Posterior reversible encephalopathy syndrome in children with kidney disease. Int Urol Nephrol 49, 1793–1800 (2017). https://doi.org/10.1007/s11255-017-1684-x

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