Posterior reversible encephalopathy syndrome (PRES) is an uncommon clinico-radiological condition that can result in severe brain injury. The pathogenesis of cerebral vasogenic edema, the hallmark of PRES, is not fully understood. Despite its name, there is substantial heterogeneity both in terms of imaging findings and outcome. Relatively little is known about PRES in kidney disease despite the clustering of risk factors including hypertension, autoimmune disease and immunosuppression. In a retrospective observational study of incident end-stage kidney disease patients in Southwest Ireland over a ten year period, we discovered five cases of PRES representing an incidence of 0.84% in this patient population. These five cases highlight the variability in clinical presentation and the potentially life-threatening nature of this condition. We provide an in-depth review of the existing literature regarding PRES in terms of its pathogenesis and heterogeneity, as well as the experience of PRES in ESKD patients. PRES appears to be rare in the ESKD population but could be under-recognized. Marked hypertension is a cardinal risk factor in this population, associated with extracellular fluid volume expansion. Neuroimaging findings can be diverse involving both anterior and posterior circulation territories. Three of the five patients described had commenced haemodialysis within four weeks of their presentation. These patients may be particularly vulnerable to microvascular brain injury, which can be devastating. This emphasises the need for clinicians to pay meticulous attention to extracellular fluid volume control during this potentially hazardous period.

Keywords:
Posterior reversible encephalopathy syndrome, End-stage kidney disease, Haemodialysis, Magnetic resonance imaging, Hypertension
1.
Bartynski WS, Boardman JF: Distinct imaging patterns and lesion distribution in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol 2007;28:1320-1327.
2.
Liman TG, Bohner G, Heuschmann PU, Endres M, Siebert E: The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study. J Neurol 2012;259:155-164.
3.
Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, et al: A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996;334:494-500.
4.
Maizlin ZV, Ghandehari H, Maizels L, Shewchuk JR, Kirby JM, Vora P, et al: Linguistic history of posterior reversible encephalopathy syndrome: mirror of developing knowledge. J Neuroimaging 2011;21:1-4.
5.
Stott VL, Hurrell MA, Anderson TJ: Reversible posterior leukoencephalopathy syndrome: a misnomer reviewed. Intern Med J 2005;35:83-90.
6.
Guyton AC, Hall JE: Overview of the circulation; biophysics of pressure, flow and resistance; in Textbook of Medical Physiology, ed 12. Philadelphia, Elsevier Saunders, 2011, pp 157-166.
7.
Guyton AC, Hall JE: Cerebral blood flow, cerebrospinal fluid, and brain metabolism; in Textbook of Medical Physiology, ed 12. Philadelphia, Elsevier Saunders, 2011, pp 743-750.
8.
Mukherjee P, McKinstry RC: Reversible posterior leukoencephalopathy syndrome: evaluation with diffusion-tensor MR imaging. Radiology 2001;219:756-765.
9.
Ay H, Buonanno FS, Schaefer PW, Le DA, Wang B, Gonzalez RG, et al: Posterior leukoencephalopathy without severe hypertension: utility of diffusion-weighted MRI. Neurology 1998;51:1369-1376.
10.
Covarrubias DJ, Luetmer PH, Campeau NG: Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images. AJNR Am J Neuroradiol 2002;23:1038-1048.
11.
Bartynski WS: Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 2008;29:1043-1049.
12.
Hefzy HM, Bartynski WS, Boardman JF, Lacomis D: Hemorrhage in posterior reversible encephalopathy syndrome: imaging and clinical features. AJNR Am J Neuroradiol 2009;30:1371-1379.
13.
Bartynski WS, Tan HP, Boardman JF, Shapiro R, Marsh JW: Posterior reversible encephalopathy syndrome after solid organ transplantation. AJNR Am J Neuroradiol 2008;29:924-930.
14.
Cruz RJ Jr, DiMartini A, Akhavanheidari M, Iacovoni N, Boardman JF, Donaldson J, et al: Posterior reversible encephalopathy syndrome in liver transplant patients: clinical presentation, risk factors and initial management. Am J Transplant 2012;12:2228-2236.
15.
Shaharir SS, Remli R, Marwan AA, Said MS, Kong NC: Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: pooled analysis of the literature reviews and report of six new cases. Lupus 2013;22:492-496.
16.
Liman TG, Bohner G, Heuschmann PU, Scheel M, Endres M, Siebert E: Clinical and radiological differences in posterior reversible encephalopathy syndrome between patients with preeclampsia-eclampsia and other predisposing diseases. Eur J Neurol 2012;19:935-943.
17.
Graham BR, Pylypchuk GB: Posterior reversible encephalopathy syndrome in an adult patient undergoing peritoneal dialysis: a case report and literature review. BMC Nephrol 2014;15:10.
18.
Ogawa A, Sugiyama H, Nakayama K, Morinaga H, Akagi S, Makino H: Reversible posterior leukoencephalopathy syndrome in a young adult patient receiving peritoneal dialysis. Perit Dial Int 2012;32:587-589.
19.
Kitamura M, Furusu A, Hirose M, Nishino T, Obata Y, Uramatsu T, et al: A case of reversible posterior leukoencephalopathy syndrome in a patient on peritoneal dialysis. Clin Exp Nephrol 2010;14:633-636.
20.
Girisgen I, Tosun A, Sonmez F, Ozsunar Y: Recurrent and atypical posterior reversible encephalopathy syndrome in a child with peritoneal dialysis. Turk J Pediatr 2010;52:416-419.
21.
Gungor O, Kircelli F, Kitis O, Asci G, Toz H, Ok E: Can strict volume control be the key for treatment and prevention of posterior reversible encephalopathy syndrome in hemodialysis patients? Hemodial Int 2013;17:107-110.
22.
Martinez-Vea A, Salvado E, Bardaji A, Gutierrez C, Ramos A, Garcia C, et al: Silent cerebral white matter lesions and their relationship with vascular risk factors in middle-aged predialysis patients with CKD. Am J Kidney Dis 2006;47:241-250.
23.
Chen CL, Lai PH, Chou KJ, Lee PT, Chung HM, Fang HC: A preliminary report of brain edema in patients with uremia at first hemodialysis: evaluation by diffusion-weighted MR imaging. AJNR Am J Neuroradiol 2007;28:68-71.
24.
Walters RJ, Fox NC, Crum WR, Taube D, Thomas DJ: Haemodialysis and cerebral oedema. Nephron 2001;87:143-147.
© 2015 S. Karger AG, Basel
2015
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.