Skip to main content

Advertisement

SpringerLink
Log in

Posterior reversible encephalopathy syndrome: characteristics, diagnostic accuracy, prognostic factors and long-term outcome in a paediatric population

  • Original article
  • Published:
Acta Neurologica Belgica Aims and scope Submit manuscript

Abstract

Posterior reversible leukoencephalopathy syndrome (PRES) is a rare entity among children, characterised by acute neurological symptoms and radiological findings. The role of clinical symptoms and neuroimaging in predicting the prognosis of PRES have not been well-characterised. A retrospective descriptive study of children with PRES, admitted to a Paediatric Intensive Care Unit during a 10-year period, was performed to describe its characteristics, compare the accuracy of computed tomography (CT) scan and MRI on diagnosis and identify prognostic factors on paediatric population. Sixteen cases were identified. Most patients (13; 81%) presented underlying disorders, including malignancies (5; 31%), chronic kidney disease (3; 19%) and post-transplant status (3; 19%). Hypertension (15; 94%) was the most common trigger. All patients had seizures, 9 patients (56%) altered state of consciousness, 8 (50%) headache. CT scan was performed in 15 patients (94%) and MRI in 13 (81%); 1 patient underwent only MRI. MRI allows the identification of new areas of vasogenic oedema and a correct diagnosis of PRES when CT scan was inconclusive. Two patients (13%) remained with neurological sequelae and one died. In two patients (13%) cognitive disorders (specific learning disorder, intellectual disability, motor tic disorder) were diagnosed during follow-up period. Clinical presentation was not statistically associated with outcome. Also, atypical neuroimaging (haemorrhagic and unilateral lesions) were not statistically related with poor neurological or cognitive outcome. However, prospective studies with a larger cohort are needed to establish prognostic factors of PRES in the paediatric population.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Legriel S, Pico F, Azoulay E (2011) Understanding posterior reversible encephalopathy syndrome. In: Vicent JL (ed) Annual update in intensive care and emergency medicine, vol 1. Springer, Berlin, pp 631–653. https://doi.org/10.1007/978-3-642-18081-1

    Chapter  Google Scholar 

  2. Hinchey J, Chaves C, Appignani B et al (1996) A reversible posterior leukoencephalopathy syndrome. N Engl J Med 334(8):494–500. https://doi.org/10.1056/NEJM199602223340803

    Article  CAS  PubMed  Google Scholar 

  3. Fischer M, Schmutzhard E (2017) Posterior reversible encephalopathy syndrome. J Neurol 264(8):1608–1616. https://doi.org/10.1007/s00415-016-8377-8

    Article  PubMed  PubMed Central  Google Scholar 

  4. Ghali MGZ, Davanzo J, Leo M, Rizk E (2019) Posterior reversible encephalopathy syndrome in pediatric patients: pathophysiology, diagnosis and management. Leuk Lymphoma 60(10):2365–2372. https://doi.org/10.1080/10428194.2019.1594210

    Article  PubMed  Google Scholar 

  5. Ganesh K, Nair RR, Kurian G, Mathew A, Sreedharan S, Paul Z (2018) Posterior reversible encephalopathy syndrome in kidney disease. Kidney Int Rep 3(2):502–507. https://doi.org/10.1016/j.ekir.2017.10.017

    Article  PubMed  Google Scholar 

  6. Musiol K, Waz S, Boroń M, Kwiatek M et al (2018) PRES in the course of hemato-oncological treatment in children. Child’s Nerv Syst 34:691–699. https://doi.org/10.1007/s00381-017-3664-y

    Article  Google Scholar 

  7. Faille LD, Fieuws S, Paesschen WV (2017) Clinical predictors and differential diagnosis of posterior reversible encephalopathy syndrome. Acta Neurol Belg 117(2):469–475. https://doi.org/10.1007/s13760-017-0750-6

    Article  PubMed  PubMed Central  Google Scholar 

  8. Shankar J, Banfield J (2016) Posterior reversible encephalopathy syndrome: a review. CAR J 68(2):147–153. https://doi.org/10.1016/j.carj.2016.08.005

    Article  Google Scholar 

  9. Siebert E, Bohner G, Endres M, Liman TG (2014) Clinical and radiological spectrum of posterior reversible encephalopathy syndrome: does age make a difference?—a retrospective comparison between adult and pediatric patients. PLoS ONE 9(12):e115073. https://doi.org/10.1371/journal.pone.0115073

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Aracki-Trenkic A, Stojanov D, Trenkic M et al (2016) Atypical presentation of posterior reversible encephalopathy syndrome: clinical and radiological characteristics in eclamptic patients. Bosn J Basic Med Sci 16(3):180–186. https://doi.org/10.17305/bjbms.2016.1201

    Article  PubMed  PubMed Central  Google Scholar 

  11. Lee VH, Wijdicks EFM, Manno EM, Rabinstein AA (2008) Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 65(2):205–210. https://doi.org/10.1001/archneurol.2007.46

    Article  PubMed  Google Scholar 

  12. Anastasopoulou S, Eriksson M, Heyman M et al (2018) Posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia: clinical characteristics, risk factors, course, and outcome of disease. Pediatr Blood Cancer. https://doi.org/10.1002/pbc.27594

    Article  PubMed  Google Scholar 

  13. Komur M, Ozgur A, Delibas A et al (2020) Posterior reversible encephalopathy syndrome (PRES) due to acute hypertension in children: 12 years single-center experience. Acta Neurol Belg. https://doi.org/10.1007/s13760-020-01423-1

    Article  PubMed  Google Scholar 

  14. Lurbe E, Agabiti-Rosei E, Cruickshank JK et al (2016) European Society of Hypertension guidelines for the management of high blood pressure in children and adolescents. J Hypertens 34(10):1887–1920. https://doi.org/10.1097/HJH.0000000000001039

    Article  CAS  PubMed  Google Scholar 

  15. Masetti R, Cordelli DM, Zama D et al (2015) PRES in children undergoing hematopoietic stem cell or solid organ transplantation. Pediatrics 135(5):890–901. https://doi.org/10.1542/peds.2014-2325

    Article  PubMed  Google Scholar 

  16. Goodwin JE, Geller DS (2011) Glucocorticoid-induced hypertension. Pediatr Nephrol 27:1059–1066. https://doi.org/10.1007/s00467-011-1928-4

    Article  PubMed  Google Scholar 

  17. Morris EB, Laningham FH, Sandlund JT, Khan RB (2007) Posterior reversible encephalopathy syndrome in children with cancer. Pediatr Blood Cancer 48(2):152–159. https://doi.org/10.1002/pbc.20703

    Article  PubMed  Google Scholar 

  18. Chen TH, Lin WC, Tseng YH, Tseng CM, Chang TT, Lin TJ (2013) Posterior reversible encephalopathy syndrome in children: case series and systematic review. J Child Neurol 28(11):1378–1386. https://doi.org/10.1177/0883073813500714

    Article  PubMed  Google Scholar 

  19. Chen TH (2020) Childhood posterior reversible encephalopathy syndrome: clinicoradiological characteristics, managements, and outcome. Front Pediatr 8:585. https://doi.org/10.3389/fped.2020.00585

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Kim SJ, Im SA, Lee JW et al (2012) Predisposing factors of posterior reversible encephalopathy syndrome in acute childhood leukemia. Pediatr Neurol 47(6):436–442. https://doi.org/10.1016/j.pediatrneurol.2012.07.011

    Article  PubMed  Google Scholar 

  21. Gamie Z, Rizwan A, Balen FG, Clarke M, Hassoon MM (2021) Posterior reversible encephalopathy syndrome in a child with cyclical vomiting and hypertension: a case report. J Med Case Rep 5:137. https://doi.org/10.1186/1752-1947-5-137

    Article  PubMed  PubMed Central  Google Scholar 

  22. Chen Z, Zhang G, Lerner A et al (2018) Risk factors for poor outcome in posterior reversible encephalopathy syndrome: systematic review and meta-analysis. Quant Imaging Med Surg 8(4):421–432. https://doi.org/10.21037/qims.2018.05.07

    Article  PubMed  PubMed Central  Google Scholar 

  23. Morris EB, Laningham FH, Sandlund JT, Khan RB (2007) Posterior reversible encephalopathy syndrome in children with cancer. Pediatr Blood Cancer 48:152–159. https://doi.org/10.1002/pbc.20703

    Article  PubMed  Google Scholar 

  24. Tambasco N, Mastrodicasa E, Salvatori C et al (2016) Prognostic factors in children with PRES and hematologic diseases. Acta Neurol Scand 134(6):474–483. https://doi.org/10.1111/ane.12570

    Article  CAS  PubMed  Google Scholar 

  25. Khan RB, Sadighi ZS, Zabrowski J, Gajjar A, Jeha S (2016) Imaging patterns and outcome of posterior reversible encephalopathy syndrome during childhood cancer treatment. Pediatr Blood Cancer 63(3):523–526. https://doi.org/10.1002/pbc.25790

    Article  CAS  PubMed  Google Scholar 

  26. Siebert E, Bohner G, Liebig T, Endres M, Liman TG (2016) Factors associated with fatal outcome in posterior reversible encephalopathy syndrome: a retrospective analysis of the Berlin PRES study. J Neurol. https://doi.org/10.1007/s00415-016-8328-4

    Article  PubMed  Google Scholar 

  27. Liman TG, Bohner G, Heuschmann PU, Endres M, Siebert E (2012) The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study. J Neurol 259:155–164. https://doi.org/10.1007/s00415-011-6152-4

    Article  CAS  PubMed  Google Scholar 

  28. Alhilali LM, Reynolds AR, Fakhran S (2014) A multi-disciplinary model of risk factors for fatal outcome in posterior reversible encephalopathy syndrome. J Neurol Sci 347:59–65. https://doi.org/10.1016/j.jns.2014.09.019

    Article  PubMed  Google Scholar 

  29. Darwish AH (2019) Posterior reversible encephalopathy syndrome in children: a prospective follow-up study. J Child Neurol. https://doi.org/10.1177/0883073819876470

    Article  PubMed  Google Scholar 

Download references

Funding

There was no funding for this study.

Author information

Authors and Affiliations

  1. Pediatric Department, Centro Hospitalar Universitário Lisboa Norte, Avenida Professor Egas Moniz, 1649-035, Lisbon, Portugal

    Catarina Marques Duarte & Ana Sofia Vilardouro

  2. Pediatric Unit, Children’s Department, Hospital de Cascais Dr. José de Almeida, Alcabideche, Portugal

    Duarte Rebelo

  3. Unit of Pediatric Neurology, Pediatric Department, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal

    Sofia Quintas

  4. Paediatric Intensive Care Unit, Pediatric Department, Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal

    Francisco Abecasis

Authors
  1. Catarina Marques Duarte
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ana Sofia Vilardouro
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Duarte Rebelo
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Sofia Quintas
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Francisco Abecasis
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by CMD, ASV and FA. The first draft of the manuscript was written by CMD and ASV and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Catarina Marques Duarte.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval retrospective studies

Due to the retrospective and observational nature of the study a formal ethical approval was waived.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Duarte, C.M., Vilardouro, A.S., Rebelo, D. et al. Posterior reversible encephalopathy syndrome: characteristics, diagnostic accuracy, prognostic factors and long-term outcome in a paediatric population. Acta Neurol Belg 122, 485–495 (2022). https://doi.org/10.1007/s13760-021-01819-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13760-021-01819-7

Keywords

Search

Navigation