Paediatric posterior reversible encephalopathy syndrome: a review of aetiologies, clinical and radiological spectrum, pathophysiology, diagnostic criteria and management

Rachna Sehgal; Archana Kashyap; Bhavna Anand; Arpita Gupta

doi:10.18203/2349-3291.ijcp20262985

Authors

Rachna Sehgal Department of Paediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Archana Kashyap Department of Paediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Bhavna Anand Department of Paediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
Arpita Gupta Department of Paediatrics, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20262985

Keywords:

PRES, Diagnosis, Genetics, Management

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a rare clinico-radiological syndrome postulated to be due to disturbed cerebral autoregulation characterized by acute onset seizures, altered sensorium, headache, visual disturbances, and typical neuroimaging findings of vasogenic oedema in parietooccipital white matter. Although originally described in adults, PRES is increasingly recognized in children, particularly in association with renal disease, hypertension, autoimmune disorders, malignancy, chemotherapy, transplantation, infections, and immune-mediated conditions. Paediatric PRES differs from adult PRES in having a higher frequency of atypical imaging findings involving brainstem, spinal cord, basal ganglia; and broader etiological diversity. Advances in neuroimaging, molecular biology, and endothelial dysfunction research have improved understanding of disease mechanisms. However, diagnostic criteria remain largely clinico-radiological. This review summarizes current evidence regarding diagnosis, typical and atypical radiological findings, aetiologies, tertiary-care experiences, pathophysiological mechanisms, emerging genetic associations, and an algorithmic approach to management.

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References

Ganzevoort W, Boterbloem K, van der Post J, Bleker O. 'Blindness out of the womb', a historical account of the first report of posterior reversible encephalopathy syndrome (PRES): choose your title well or your findings will be neglected. BJOG. 2020;127(1):37-8. DOI: https://doi.org/10.1111/1471-0528.16004

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(8):494-500. DOI: https://doi.org/10.1056/NEJM199602223340803

Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of FLAIR imaging in the detection of cortical and subcortical lesions. Am J Neuroradiol. 2000;21:1199-206.

Sharma S, Gupta R, Sehgal R, Aggarwal KC. Atypical presentation of posterior reversible encephalopathy: in a child with bilateral grade IV vesicoureteric reflux. J Trop Pediatr. 2014;60(4):331-3. DOI: https://doi.org/10.1093/tropej/fmu019

Thavamani A, Umapathi KK, Puliyel M, Super D, Allareddy V, Ghori A. Epidemiology, Comorbidities, and Outcomes of Posterior Reversible Encephalopathy Syndrome in Children in the United States. Pediatr Neurol. 2020;103:21-6. DOI: https://doi.org/10.1016/j.pediatrneurol.2019.07.007

He M, Gillespie A. Posterior Reversible Encephalopathy Syndrome in Chronic Kidney Disease: Incidence, Outcomes, and Risk Factors in a National Cohort. Kidney Med. 2025;7(7):101022. DOI: https://doi.org/10.1016/j.xkme.2025.101022

Orlando C, Milani GP, Simonetti GD, Goeggel Simonetti B, Lava SAG, Wyttenbach R, et al. Posterior reversible leukoencephalopathy syndrome associated with acute postinfectious glomerulonephritis: systematic review. Pediatr Nephrol. 2022;37(4):833-41. DOI: https://doi.org/10.1007/s00467-021-05244-z

Yamada A, Atsumi M, Tashiro A, Hiraiwa T, Ueda N. Recurrent posterior reversible encephalopathy syndrome in nephrotic syndrome: case report and review of the literature. Clin Nephrol. 2012;78(5):406-11. DOI: https://doi.org/10.5414/CN106976

Youssef D, Fawzy F. Posterior reversible encephalopathy syndrome in a child with steroid sensitive nephrotic syndrome. Arab J Nephrol Transplant. 2012;5(3):163-6.

Zhang Y, Zhou J, Chen Y. Posterior reversible encephalopathy syndrome in a child with steroid-resistant nephrotic syndrome: a case report and review of literature. Int J Clin Exp Pathol. 2014;7(7):4433-7.

Karpman D, Loos S, Tati R, Arvidsson I. Haemolytic Uraemic Syndrome. J Intern Med. 2017;281:123-48. DOI: https://doi.org/10.1111/joim.12546

Tonkovic U, Bogicevic M, Manzar A, Andrejic N, Sic A, Atanaskovic M, et al. Neurological Manifestations of Hemolytic Uremic Syndrome: A Comprehensive Review. Brain Sci. 2025;15(7):717. DOI: https://doi.org/10.3390/brainsci15070717

Manadan A, Kambhatla S, Gauto-Mariotti E, Okoli C, Block JA. Rheumatic diseases associated with posterior reversible encephalopathy syndrome. J Clin Rheumatol. 2021;27:4. DOI: https://doi.org/10.1097/RHU.0000000000001470

Ramanathan S, Subramani V, Kembhavi S, Prasad M, Roy Moulik N, Dhamne C, et al. Clinical features, predictors and outcome of posterior reversible encephalopathy syndrome (PRES) in children with hematolymphoid malignancies. Childs Nerv Syst. 2022;38(9):1689-98. DOI: https://doi.org/10.1007/s00381-022-05557-w

Teive HA, Brandi IV, Camargo CH, Bittencourt MA, Bonfim CM, Friedrich ML, et al. Reversible posterior leucoencephalopathy syndrome associated with bone marrow transplantation. Arq Neuropsiquiatr. 2001;59(3-B):784-9. DOI: https://doi.org/10.1590/S0004-282X2001000500024

Kaur G, Ashraf I, Peck MM, Maram R, Mohamed A, Crespo DO, et al. Chemotherapy and Immunosuppressant Therapy-Induced Posterior Reversible Encephalopathy Syndrome. Cureus. 2020;12(10):e11163. DOI: https://doi.org/10.7759/cureus.11163

How J, Blattner M, Fowler S, Wang-Gillam A, Schindler SE. Chemotherapy-associated Posterior Reversible Encephalopathy Syndrome: A Case Report and Review of the Literature. Neurologist. 2016;21(6):112-7. DOI: https://doi.org/10.1097/NRL.0000000000000105

Wake K, Imataka G, Ohnishi T, Kikuchi J, Hoshiyama E, Ono K, et al. A Case-Study of a Child with Reversible Posterior Leukoencephalopathy Syndrome (RPLS) Associated with Severe Burns throughout the Body. Iran J Public Health. 2020;49(7):1390-1. DOI: https://doi.org/10.18502/ijph.v49i7.3600

He X, Shi D, Long C, Yang J, Tian J. Posterior reversible encephalopathy syndrome in a child after burns: A case report and literature review. SAGE Open Med Case Rep. 2023;11:2050313X231157988. DOI: https://doi.org/10.1177/2050313X231157988

Barba L, Carrubba C, Spindler K, Weise CM, Sachs T, Foschi M, et al. Posterior reversible encephalopathy syndrome associated with antibiotic therapy: a case report and systematic review. Neurol Sci. 2024;45(9):4151-9. DOI: https://doi.org/10.1007/s10072-024-07545-1

Aydin K, Elmas S, Guzes EA. Reversible posterior leukoencephalopathy and Adie's pupil after measles vaccination. J Child Neurol. 2006;21(6):525-7. DOI: https://doi.org/10.1177/08830738060210060901

Srichawla BS, Fang T, Kipkorir V, Garcia-Dominguez MA. Reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome following vaccination: analysis of the VAERS database and systematic review. Ann Med Surg (Lond). 2023;86(3):1251-60. DOI: https://doi.org/10.1097/MS9.0000000000001407

Sano H, Yamazaki T, Kudo E, Hatakeyama K, Ito T. Posterior reversible encephalopathy syndrome associated with mild COVID-19 infection in a 9-year-old child: A case report and literature review. IDCases. 2023;31:e01699. DOI: https://doi.org/10.1016/j.idcr.2023.e01699

Palliyaguru T, Ruwanpathirana P, Aravinthan M, Priyankara D, Weeratunga P. Posterior reversible encephalopathy syndrome in severe leptospirosis: A case report. IDCases. 2025;41:e02330. DOI: https://doi.org/10.1016/j.idcr.2025.e02330

Christofidis C, Anastasiou A, Krasnikova E, Mandros C, Potolidis E. Posterior reversible encephalopathy syndrome as a complication of bacterial meningitis. Hippokratia. 2019;23(3):131-4.

Lee DJ, Kim JS. Rotavirus Infection-Associated Posterior Reversible encephalopathy Syndrome. Ann Child Neurol. 2019;27(2):60-2. DOI: https://doi.org/10.26815/acn.2019.00101

Ito Y, Toyama K, Honda A, Nakazaki K, Arai S, Kurokawa M. Posterior reversible encephalopathy syndrome concurrent with human herpesvirus-6B encephalitis after allogeneic hematopoietic stem cell transplantation. J Infect Chemother. 2019;26(2):265-8. DOI: https://doi.org/10.1016/j.jiac.2019.07.016

Chen TH. Childhood Posterior Reversible Encephalopathy Syndrome: Clinicoradiological Characteristics, Managements, and Outcome. Front Pediatr. 2020;8:585. DOI: https://doi.org/10.3389/fped.2020.00585

Mani SH, Shivaprasad SM, Umesh L. A rare presentation of lupus nephritis flare up with posterior reversible leucoencephalopathy. J Clin Diagn Res. 2016;10:4. DOI: https://doi.org/10.7860/JCDR/2016/14770.7038

Bhangu JK, Javed K, Manshahia PK, Nahar S, Kanda S, Chatha U, et al. The Association of Hypertension With Posterior Reversible Encephalopathy Syndrome in Systemic Lupus Erythematosus Patients: A Systematic Review. Cureus. 2023;15(12):e50620. DOI: https://doi.org/10.7759/cureus.50620

Radiopaedia. Posterior reversible encephalopathy syndrome. 2026. Available at: https://radiopaedia. org/articles/posterior-reversible-encephalopathy-syndrome-1. Accessed on 06 July 2026.

Bartynski W, Boardman J. Distinct Imaging Patterns and Lesion Distribution in Posterior Reversible Encephalopathy Syndrome. AJNR Am J Neuroradiol. 2007;28(7):1320-7. DOI: https://doi.org/10.3174/ajnr.A0549

Kaufmann J, Buecke P, Meinel T, Beyeler M, Scutelnic A, Kaesmacher J, et al. Frequency of ischaemic stroke and intracranial haemorrhage in patients with reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES) - A systematic review. Eur J Neurol. 2024;31(5):e16246. DOI: https://doi.org/10.1111/ene.16246

Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. 2015;14:914-25. DOI: https://doi.org/10.1016/S1474-4422(15)00111-8

Chardain A, Mesnage V, Alamowitch S, Bourdain F, Crozier S, Lenglet T, et al. Posterior reversible encephalopathy syndrome (PRES) and hypomagnesemia: a frequent association? Rev Neurol. 2016;172(6–7):384-8. DOI: https://doi.org/10.1016/j.neurol.2016.06.004

Gao B, Yu BX, Li RS, Zhang G, Xie HZ, Liu FL, et al. Cytotoxic edema in posterior reversible encephalopathy syndrome: correlation of MRI features with serum albumin levels. AJNR Am J Neuroradiol. 2015;36(10):1884-9. DOI: https://doi.org/10.3174/ajnr.A4379

Largeau B, Bergeron S, Auger F, Salmon Gandonnière C, Jonville-Béra AP, Ehrmann S, et al. Experimental Models of Posterior Reversible Encephalopathy Syndrome: A Review From Pathophysiology to Therapeutic Targets. Stroke. 2024;55(2):484-93. DOI: https://doi.org/10.1161/STROKEAHA.123.044533

ClinicalTrial.Gov. The Arginine Vasopressin aXis as a Potential Therapeutic Target for Posterior REverSible Encephalopathy SyndromE (XPRESSE). 2022. Available at: https://clinicaltrials.gov/ct2/ show/NCT04950270. Accessed on 15 June 2026.

Fischer M, Schmutzhard E. Posterior reversible encephalopathy syndrome. J Neurol. 2017;264(8):1608-16. DOI: https://doi.org/10.1007/s00415-016-8377-8

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(5):427-32. DOI: https://doi.org/10.4065/mcp.2009.0590

Gupta V, Bhatia V, Khandelwal N, Singh P, Singhi P. Imaging Findings in Pediatric Posterior Reversible Encephalopathy Syndrome (PRES): 5 Years of Experience From a Tertiary Care Center in India. J Child Neurol. 2016;31(9):1166-73. DOI: https://doi.org/10.1177/0883073816643409

Gaddam S, Kodandapani R, Mani N, Jayaraman D, Abirami BN. Posterior reversible encephalopathy syndrome in immunocompromised children - A single-center study from South India. J Neurosci Rural Pract. 2024;15(2):365-9. DOI: https://doi.org/10.25259/JNRP_390_2023

Emeksiz S, Kutlu NO, Çaksen H, Alkan G, Yıkmaz HŞ, Tokgöz H. Posterior reversible encephalopathy syndrome in children: a case series. Turk Pediatri Ars. 2016;51(4):217-20. DOI: https://doi.org/10.5152/TurkPediatriArs.2016.2192

de Laat P, Te Winkel ML, Devos AS, Catsman-Berrevoets CE, Pieters R, van den Heuvel-Eibrink MM. Posterior reversible encephalopathy syndrome in childhood cancer. Ann Oncol. 2011;22(2):472-8. DOI: https://doi.org/10.1093/annonc/mdq382

Srichawla BS, Kaur T, Singh H. Corticosteroids in posterior reversible encephalopathy syndrome: Friend or foe? A systematic review. World J Clin Cases. 2025;13(12):98768. DOI: https://doi.org/10.12998/wjcc.v13.i12.98768

Meyer M, Niemöller U, Stein T, Schmetsdorf S, Arnold A, El-Sheik M, et al. Positive Effect of Steroids in Posterior Reversible Encephalopathy Syndrome. Case Rep Neurol. 2019;11(2):173-7. DOI: https://doi.org/10.1159/000500410

Gavrilovici C, Miron I, Voroneanu L, Bădărau S, Stârcea M. Posterior reversible encephalopathy syndrome in children with kidney disease. Int Urol Nephrol. 2017;49(10):1793-800. DOI: https://doi.org/10.1007/s11255-017-1684-x

Triplett JD, Kutlubaev MA, Kermode AG, Hardy T. Posterior reversible encephalopathy syndrome (PRES): diagnosis and management. Pract Neurol. BMJ Publishing Group Ltd. 2022. DOI: https://doi.org/10.1136/practneurol-2021-003194

Hinduja A. Posterior reversible encephalopathy syndrome: clinical features and outcome. Front Neurol. 2020;11:71. DOI: https://doi.org/10.3389/fneur.2020.00071

Gewirtz AN, Gao V, Parauda SC, Robbins MS. Posterior reversible encephalopathy syndrome. Curr Pain Headache Rep. 2021;25:19. DOI: https://doi.org/10.1007/s11916-020-00932-1

Nada MG, Libda YI, Gohary MM, Dessouky R. Pediatric posterior reversible encephalopathy syndrome: Can MR imaging features predict outcomes in non-oncologic patients? Eur J Radiol. 2024;170:111214. DOI: https://doi.org/10.1016/j.ejrad.2023.111214