Abstract
Introduction: Posterior reversible encephalopathy syndrome (PRES) is a neurovascular disorder characterized by vasogenic edema, most often affecting the parieto-occipital lobes. Although typically reversible, some patients develop lasting neurological sequelae such as cognitive or visual impairment and epilepsy. This review summarizes the current understanding of PRES beyond its classical association with hypertension, emphasizing emerging triggers, mechanisms, and updates in management.
Methods: A structured narrative review was conducted in accordance with the Scale for the Assessment of Narrative Review Articles (SANRA) framework. Literature was systematically searched in PubMed, Web of Science, and Google Scholar for studies published between January 1996 and June 2025. Fifty-nine relevant publications, including original studies and reviews, were synthesized to integrate evidence on presentation, imaging, pathophysiology, genetics, and management.
Results: PRES can be triggered by a broad spectrum of hypertensive and non-hypertensive conditions, such as renal dysfunction, eclampsia, systemic lupus erythematosus, sepsis, and exposure to immunosuppressive agents (e.g., tacrolimus, cyclosporine, rituximab). The syndrome involves multifactorial mechanisms, including endothelial dysfunction, blood–brain barrier disruption, and dysregulated cerebral autoregulation. Emerging data indicate that genetic polymorphisms in endothelial nitric oxide synthase (eNOS), vascular endothelial growth factor (VEGF), and aquaporin genes may influence individual susceptibility, although causality remains unconfirmed.
Conclusion: PRES is a multifactorial syndrome that extends beyond hypertension. Early recognition, prompt neuroimaging, and targeted management of underlying triggers are critical for improving outcomes. Future studies should focus on clarifying genetic associations, standardizing diagnostic criteria for atypical cases, and optimizing therapeutic strategies through personalized medicine approaches.
References
1. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, Wang A, Pessin MS, Lamy C, Mas JL, Caplan LR. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996: 494 [PMID: 8559202, https://doi.org/10.1056/NEJM199602223340803]
2. Lee VH, Wijdicks EF, Manno EM, Rabinstein AA. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol. 2008: 205 [PMID: 18268188, https://doi.org/10.1001/archneurol.2007.46]
3. Zou LP, Liu LY, Li H, Wang YY, Liu Y, Chen J, Hu LY, Liu MJ, Zhang MN, Lu Q, Ma SF. Establishment and utility assessment of posterior reversible encephalopathy syndrome early warning scoring (PEWS) scale establishment and utility assessment of PEWS scale. BMC Neurol. 2019: 30 [PMID: 30791893, https://doi.org/10.1186/s12883-019-1247-0]
4. Aracki-Trenkic A, Stojanov D, Trenkic M, Radovanovic Z, Ignjatovic J, Ristic S, Trenkic-Bozinovic M. Atypical presentation of posterior reversible encephalopathy syndrome: Clinical and radiological characteristics in eclamptic patients. Bosn J Basic Med Sci. 2016: 180 [PMID: 27322924, https://doi.org/10.17305/bjbms.2016.1201]
5. Samara A, Berry B, Ghannam M. Posterior reversible encephalopathy syndrome with isolated infratentorial involvement: A case report. Radiol Case Rep. 2019: 576 [PMID: 30886669, https://doi.org/10.1016/j.radcr.2019.02.009]
6. Mackay DD, Zepeda Garcia R, Galetta SL, Prasad S. Periodic alternating gaze deviation and nystagmus in posterior reversible encephalopathy syndrome. Neurol Clin Pract. 2014: 482 [PMID: 29443162, https://doi.org/10.1212/CPJ.0000000000000056]
7. Abraham P, Longardner K, Chen P, Huisa B, Handwerker J. Case 279: Central-Variant Posterior Reversible Encephalopathy Syndrome. Radiology. 2020: 239 [PMID: 32539624, https://doi.org/10.1148/radiol.2020181547]
8. Anderson RC, Patel V, Sheikh-Bahaei N, Liu CSJ, Rajamohan AG, Shiroishi MS, Kim PE, Go JL, Lerner A, Acharya J. Posterior Reversible Encephalopathy Syndrome (PRES): Pathophysiology and Neuro-Imaging. Front Neurol. 2020: 463 [PMID: 32612567, https://doi.org/10.3389/fneur.2020.00463]
9. Hartmann H. Posterior Reversible Encephalopathy: An Epileptic Syndrome? Neuropediatrics. 2017: 64 [PMID: 28282666, https://doi.org/10.1055/s-0037-1600517]
10. Zelaya JE, Al-Khoury L. Posterior Reversible Encephalopathy Syndrome. StatPearls. Treasure Island (FL): StatPearls Publishing
Copyright © 2025, StatPearls Publishing LLC.; 2025.
11. 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: 427 [PMID: 20435835, https://doi.org/10.4065/mcp.2009.0590]
12. Fischer M, Schmutzhard E. Posterior reversible encephalopathy syndrome. J Neurol. 2017: 1608 [PMID: 28054130, https://doi.org/10.1007/s00415-016-8377-8]
13. Hobson EV, Craven I, Blank SC. Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness. Perit Dial Int. 2012: 590 [PMID: 23212858, https://doi.org/10.3747/pdi.2012.00152]
14. Otite FO, Patel SD, Anikpezie N, Hoffman H, Beutler T, Akano EO, Aneni E, Lamikanra O, Osondu C, Wee C, Burke D, Albright KC, Latorre JG, Mejico L, Khandelwal P, Chaturvedi S. Demographic Disparities in the Incidence, Clinical Characteristics, and Outcome of Posterior Reversible Encephalopathy Syndrome in the United States. Neurology. 2023: e1554 [PMID: 37487751, https://doi.org/10.1212/WNL.0000000000207604]
15. McKinney AM, Short J, Truwit CL, McKinney ZJ, Kozak OS, SantaCruz KS, Teksam M. Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings. AJR Am J Roentgenol. 2007: 904 [PMID: 17885064, https://doi.org/10.2214/AJR.07.2024]
16. Harirchian MH, Ghaffarpour M, Tabaeizadeh M, Siroos B. Immunosuppressive Drugs, an Emerging Cause of Posterior Reversible Encephalopathy Syndrome: Case Series. J Stroke Cerebrovasc Dis. 2015: e191 [PMID: 26082344, https://doi.org/10.1016/j.jstrokecerebrovasdis.2015.02.014]
17. Triplett JD, Kutlubaev MA, Kermode AG, Hardy T. Posterior reversible encephalopathy syndrome (PRES): diagnosis and management. Pract Neurol. 2022: 183 [PMID: 35046115, https://doi.org/10.1136/practneurol-2021-003194]
18. Odoma VA, Zahedi I, Haq H, Lopez Pantoja SC, Onyejide EC, Rahman F. Malignant Hypercalcemia: A Rare Etiology of Posterior Reversible Encephalopathy Syndrome. Cureus. 2023: e41229 [PMID: 37529521, https://doi.org/10.7759/cureus.41229]
19. Nakamura Y, Sugino M, Tsukahara A, Nakazawa H, Yamamoto N, Arawaka S. Posterior reversible encephalopathy syndrome with extensive cytotoxic edema after blood transfusion: a case report and literature review. BMC Neurol. 2018: 190 [PMID: 30419835, https://doi.org/10.1186/s12883-018-1194-1]
20. Dev N, Kumar R, Kumar P, Kumawat A. Hyperglycemia-induced posterior reversible encephalopathy syndrome: A rare cause of reversible blindness. J Family Med Prim Care. 2019: 3431 [PMID: 31742185, https://doi.org/10.4103/jfmpc.jfmpc_695_19]
21. Tawati DA, Chan WS. A systematic review of posterior reversible encephalopathy syndrome in pregnant women with severe preeclampsia and eclampsia. Obstet Med. 2023: 236 [PMID: 38074208, https://doi.org/10.1177/1753495X221150302]
22. Keepanasseril A, Nayak D, Bojja V, Gupta A, Chakkalakkoombil SV, Nair PP, Thilaganathan B. Risk factors for posterior reversible encephalopathy syndrome in hypertensive pregnant women presenting with seizures. Neurol Sci. 2022: 3839 [PMID: 35091885, https://doi.org/10.1007/s10072-021-05847-2]
23. Chao AS, Chen YL, Chang YL, Chao A, Su SY, Wang TH. Severe pre-eclamptic women with headache: is posterior reversible encephalopathy syndrome an associated concurrent finding? BMC Pregnancy Childbirth. 2020: 336 [PMID: 32487027, https://doi.org/10.1186/s12884-020-03017-4]
24. Hu D, Xiong J, Zha Y, Zhang Z. Posterior reversible encephalopathy syndrome complicated with subarachnoid hemorrhage in an eclamptic pregnant patient: case report. BMC Neurol. 2018: 182 [PMID: 30390663, https://doi.org/10.1186/s12883-018-1186-1]
25. Balcerac A, Bihan K, Psimaras D, Lebrun-Vignes B, Salem JE, Weiss N. Drugs associated with posterior reversible encephalopathy syndrome, a worldwide signal detection study. J Neurol. 2023: 975 [PMID: 36329183, https://doi.org/10.1007/s00415-022-11450-y]
26. Mustafa KN, Qasem U, Al-Ryalat NT, Bsisu IK. Rituximab-associated posterior reversible encephalopathy syndrome. Int J Rheum Dis. 2019: 160 [PMID: 30398015, https://doi.org/10.1111/1756-185X.13427]
27. Mogawer M-S, El-Shazly M, Salah A, Abdel-Maqsod A, Abdelaziz O, Abdel-Ghani A, Essam M, Abdellatif AA. Tacrolimus-induced posterior reversible encephalopathy syndrome presenting as left upper limb monoplegia, convulsions, and sudden blindness: case report. Egyptian Liver Journal. 2020: https://doi.org/10.1186/s43066-020-00064-6]
28. Bartynski WS, Tan HP, Boardman JF, Shapiro R, Marsh JW. Posterior reversible encephalopathy syndrome after solid organ transplantation. AJNR Am J Neuroradiol. 2008: 924 [PMID: 18272559, https://doi.org/10.3174/ajnr.A0960]
29. Khan IR, Pai V, Mundada P, Sitoh YY, Purohit B. Detecting the Uncommon Imaging Manifestations of Posterior Reversible Encephalopathy Syndrome (PRES) in Adults: a Comprehensive Illustrated Guide for the Trainee Radiologist. Curr Probl Diagn Radiol. 2022: 98 [PMID: 33257096, https://doi.org/10.1067/j.cpradiol.2020.11.002]
30. Manadan A, Kambhatla S, Gauto-Mariotti E, Okoli C, Block JA. Rheumatic Diseases Associated With Posterior Reversible Encephalopathy Syndrome. J Clin Rheumatol. 2021: e391 [PMID: 32604240, https://doi.org/10.1097/RHU.0000000000001470]
31. Merayo-Chalico J, Apodaca E, Barrera-Vargas A, Alcocer-Varela J, Colunga-Pedraza I, Gonzalez-Patino A, Arauz A, Abud-Mendoza C, Martinez-Martinez M, Gomez-Martin D. Clinical outcomes and risk factors for posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a multicentric case-control study. J Neurol Neurosurg Psychiatry. 2016: 287 [PMID: 25804426, https://doi.org/10.1136/jnnp-2014-310145]
32. Kadoh Y, Yoshino J, Oka T, Itoga K, Hanada M, Niino D, Nagai A, Ichinose K, Kanda T. A case of posterior and reversible encephalopathy syndrome in a patient previously undiagnosed with lupus nephritis. CEN Case Rep. 2025: 328 [PMID: 39918729, https://doi.org/10.1007/s13730-025-00973-8]
33. Garcia-Grimshaw M, Dominguez-Moreno R, Valdes-Ferrer SI. Posterior Reversible Encephalopathy Syndrome: An Underrecognized Manifestation of Systemic Lupus Erythematosus. Neurohospitalist. 2020: 234 [PMID: 32549950, https://doi.org/10.1177/1941874419889467]
34. Orhun G, Sencer S, Tuzun E, Bebek N, Ergin Ozcan P, Barburoglu M, Gunver MG, Esen F. Posterior Reversible Encephalopathy in Sepsis-Associated Encephalopathy: Experience from a Single Center. Neurocrit Care. 2022: 372 [PMID: 35133605, https://doi.org/10.1007/s12028-021-01433-8]
35. Boccio E, Mastroianni F, Slesinger T. Posterior Reversible Encephalopathy Syndrome in a Patient with Septic Shock: A Case Report. Clin Pract Cases Emerg Med. 2023: 153 [PMID: 37595316, https://doi.org/10.5811/cpcem.1461]
36. Bartynski WS, Boardman JF, Zeigler ZR, Shadduck RK, Lister J. Posterior reversible encephalopathy syndrome in infection, sepsis, and shock. AJNR Am J Neuroradiol. 2006: 2179 [PMID: 17110690,
37. Peng X, Luo Z, He S, Zhang L, Li Y. Blood-Brain Barrier Disruption by Lipopolysaccharide and Sepsis-Associated Encephalopathy. Front Cell Infect Microbiol. 2021: 768108 [PMID: 34804998, https://doi.org/10.3389/fcimb.2021.768108]
38. Hinduja A. Posterior Reversible Encephalopathy Syndrome: Clinical Features and Outcome. Front Neurol. 2020: 71 [PMID: 32117030, https://doi.org/10.3389/fneur.2020.00071]
39. Roth C, Ferbert A. Posterior reversible encephalopathy syndrome: long-term follow-up. J Neurol Neurosurg Psychiatry. 2010: 773 [PMID: 19955114, https://doi.org/10.1136/jnnp.2009.189647]
40. Wu Q, Marescaux C, Wolff V, Jeung MY, Kessler R, Lauer V, Chen Y. Tacrolimus-associated posterior reversible encephalopathy syndrome after solid organ transplantation. Eur Neurol. 2010: 169 [PMID: 20699617, https://doi.org/10.1159/000319032]
41. Zeeman GG. Neurologic complications of pre-eclampsia. Semin Perinatol. 2009: 166 [PMID: 19464507, https://doi.org/10.1053/j.semperi.2009.02.003]
42. Gao B, Lyu C, Lerner A, McKinney AM. Controversy of posterior reversible encephalopathy syndrome: what have we learnt in the last 20 years? J Neurol Neurosurg Psychiatry. 2018: 14 [PMID: 28794149, https://doi.org/10.1136/jnnp-2017-316225]
43. McKinney AM, Sarikaya B, Gustafson C, Truwit CL. Detection of microhemorrhage in posterior reversible encephalopathy syndrome using susceptibility-weighted imaging. AJNR Am J Neuroradiol. 2012: 896 [PMID: 22241378, https://doi.org/10.3174/ajnr.A2886]
44. Bartynski WS. Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol. 2008: 1036 [PMID: 18356474, https://doi.org/10.3174/ajnr.A0928]
45. Largeau B, Le Tilly O, Sautenet B, Salmon Gandonniere C, Barin-Le Guellec C, Ehrmann S. Arginine Vasopressin and Posterior Reversible Encephalopathy Syndrome Pathophysiology: the Missing Link? Mol Neurobiol. 2019: 6792 [PMID: 30924075, https://doi.org/10.1007/s12035-019-1553-y]
46. Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. 2015: 914 [PMID: 26184985, https://doi.org/10.1016/S1474-4422(15)00111-8]
47. Rao J, Chen Y, Chen X, Wu R, Luo S, Lin Q, Dong Z, Huang J. The gene polymorphisms of eNOS and MTHFR modulates the development of preeclampsia in Han population. Heliyon. 2023: e22223 [PMID: 38076047, https://doi.org/10.1016/j.heliyon.2023.e22223]
48. Sorani MD, Zador Z, Hurowitz E, Yan D, Giacomini KM, Manley GT. Novel variants in human Aquaporin-4 reduce cellular water permeability. Hum Mol Genet. 2008: 2379 [PMID: 18511455, https://doi.org/10.1093/hmg/ddn138]
49. Matiello M, Muralidharan R, Sun D, Rabinstein AA, Weinshenker BG. Posterior reversible encephalopathy syndrome is not associated with mutations in aquaporin-4. Neurol Genet. 2015: e19 [PMID: 27066556, https://doi.org/10.1212/NXG.0000000000000013]
50. Magana SM, Matiello M, Pittock SJ, McKeon A, Lennon VA, Rabinstein AA, Shuster E, Kantarci OH, Lucchinetti CF, Weinshenker BG. Posterior reversible encephalopathy syndrome in neuromyelitis optica spectrum disorders. Neurology. 2009: 712 [PMID: 19237699, https://doi.org/10.1212/01.wnl.0000343001.36493.ae]
51. Kamo H, Ueno Y, Sugiyama M, Miyamoto N, Yamashiro K, Tanaka R, Yokoyama K, Hattori N. Pontine hemorrhage accompanied by neuromyelitis optica spectrum disorder. J Neuroimmunol. 2019: 19 [PMID: 30769213, https://doi.org/10.1016/j.jneuroim.2019.01.020]
52. Sudan S, Kaur N, Anand S, Upadhyaya A, Taneja R. Posterior Reversible Encephalopathy Syndrome (PRES) in Systemic Lupus Erythematosus: A Rare Presentation. Cureus. 2022: e31376 [PMID: 36514640, https://doi.org/10.7759/cureus.31376]
53. Merayo-Chalico J, Barrera-Vargas A, Juarez-Vega G, Alcocer-Varela J, Arauz A, Gomez-Martin D. Differential serum cytokine profile in patients with systemic lupus erythematosus and posterior reversible encephalopathy syndrome. Clin Exp Immunol. 2018: 165 [PMID: 29271478, https://doi.org/10.1111/cei.13095]
54. Swart RM, Hoorn EJ, Betjes MG, Zietse R. Hyponatremia and inflammation: the emerging role of interleukin-6 in osmoregulation. Nephron Physiol. 2011: 45 [PMID: 21196778, https://doi.org/10.1159/000322238]
55. Song T, Rao Z, Tan Q, Qiu Y, Liu J, Huang Z, Wang X, Lin T. Calcineurin Inhibitors Associated Posterior Reversible Encephalopathy Syndrome in Solid Organ Transplantation: Report of 2 Cases and Literature Review. Medicine (Baltimore). 2016: e3173 [PMID: 27057842, https://doi.org/10.1097/MD.0000000000003173]
56. Parasher A, Jhamb R. Posterior reversible encephalopathy syndrome (PRES): presentation, diagnosis and treatment. Postgrad Med J. 2020: 623 [PMID: 32467104, https://doi.org/10.1136/postgradmedj-2020-137706]
57. J. F. Posterior Reversible Encephalopathy Syndrome (PRES) - EMCRIT Project. 2022 [cited 2025 11/16/2025]. Available from: https://emcrit.org/ibcc/pres.
58. Catherine C, Yanta C, Saand AR, Pilato M, Chou SH. Pearls & Oy-sters: The dangers of PRES: An atypical case with life-threatening presentation. Neurology. 2019: e282 [PMID: 30643036, https://doi.org/10.1212/WNL.0000000000006775]
59. Datar S, Singh T, Rabinstein AA, Fugate JE, Hocker S. Long-term risk of seizures and epilepsy in patients with posterior reversible encephalopathy syndrome. Epilepsia. 2015: 564 [PMID: 25690439, https://doi.org/10.1111/epi.12933]

This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2025 Mohamed Fawzi Hemida, MD, Pranjal Kumar Singh, MBBS, Alyaa Ahmed Ibrahim, MD, Ahmed Harb, MD, Sai Lokesh Moraboina, MD, Geethanjali Prakash, MBBS, Esraa M. Soliman, MBBS, Arun Kumar Maloth, MBBS, Huda Adnan, MBBS, Ravi Virani, MBBS, Sanchit Shailendra Chouksey, MBBS, MD, DM

