Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiologic entity, typically manifesting as reversible neurological symptoms and signs of white matter edema on magnetic resonance imaging. PRES has been widely described in adults. Studies of PRES in children are mostly limited to case series and case controls.
Retrospective chart review of patients under 21 years with PRES admitted at a tertiary children’s hospital from 2011 to 2016. They were compared to controls matched for age and mortality risk using the Pediatric Index of Mortality-2 score.
Sixteen cases of PRES were identified in 13 patients (ages 5–17 years, 46% male). PRES presented with altered mental status (75%), seizures (77%), headache (31%), and vision changes (23%). In patients who recovered (n = 11), median days to symptom resolution was three (range 1–8). PRES patients had a higher mortality rate (15% vs. 5%, p < 0.05) and higher mean length of stay (13.1 vs. 4.6 days) and were more likely to have autoimmune disease (p < 0.05), immunosuppression (p < 0.05), and anemia (p < 0.05). No PRES patients were diagnosed with epilepsy by last known follow-up, and all of whom had been started on an antiepileptic drug were discontinued within 13 months. Sepsis was suspected in 53% of PRES patients and 59% of controls (p = 1.00). All PRES patients had stage II hypertension, versus 41% of controls (p < 0.05). Average creatinine in PRES was 2.35 mg/dL compared to 0.90 mg/dL in controls (p < 0.05). PRES patients had lower serum calcium (p < 0.05). After correcting for albumin, no association between PRES and hypocalcemia remained. PRES patients had a higher length of stay (13.1 vs. 4.6 days, p < 0.05) and mortality rate (15% vs. 3%, p < 0.05).
Immunosuppression, autoimmune disease, renal insufficiency, anemia, and hypertension are associated with PRES after controlling for mortality risk in critically ill children. There was no association between corrected serum calcium and sepsis with PRES.
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Altered mental status
Diastolic blood pressure
Mean arterial pressure
Magnetic resonance imaging
Magnetic resonance venogram
Pediatric intensive care unit
Pediatric Index of Mortality-2
Posterior reversible encephalopathy syndrome
Systolic blood pressure
Systemic lupus erythematous
Pediatric cerebral performance category
Pediatric overall performance category
Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334:494–500.
Stevens CJ, Heran MK. The many faces of posterior reversible encephalopathy syndrome. Br J Radiol. 2012;85:1566–75.
Yamamoto H, Natsume J, Kidokoro H, et al. Clinical and neuroimaging findings in children with posterior reversible encephalopathy syndrome. Eur J Paediatr Neurol. 2015;19:672–8.
Akin F, Kilicaslan C, Solak ES, Uzun M, Aygun S, Arslan S. Posterior reversible encephalopathy syndrome in children: report of three cases. Childs Nerv Syst. 2014;30:535–40.
Endo A, Fuchigami T, Hasegawa M, et al. Posterior reversible encephalopathy syndrome in childhood: report of four cases and review of the literature. Pediatr Emerg Care. 2012;28:153–7.
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;31:1166–73.
Muscal E, Traipe E, de Guzman MM, Myones BL, Brey RL, Hunter JV. MR imaging findings suggestive of posterior reversible encephalopathy syndrome in adolescents with systemic lupus erythematosus. Pediatr Radiol. 2010;40:1241–5.
Yamada A, Ueda N. Age and gender may affect posterior reversible encephalopathy syndrome in renal disease. Pediatr Nephrol. 2012;27:277–83.
Ergun T, Lakadamyali H, Yilmaz A. Recurrent posterior reversible encephalopathy syndrome in a hypertensive patient with end-stage renal disease. Diagn Interv Radiol. 2008;14:182–5.
Onder AM, Lopez R, Teomete U, et al. Posterior reversible encephalopathy syndrome in the pediatric renal population. Pediatr Nephrol. 2007;22:1921–9.
Daniel NJ, Hernandez CL, Walker RA. Recurrent posterior reversible encephalopathy syndrome in a pediatric patient with end-stage renal disease. J Emerg Med. 2014;46:e39–42.
Hagemann G, Ugur T, Witte OW, Fitzek C. Recurrent posterior reversible encephalopathy syndrome (PRES). J Hum Hypertens. 2004;18:287–9.
Won SC, Kwon SY, Han JW, Choi SY, Lyu CJ. Posterior reversible encephalopathy syndrome in childhood with hematologic/oncologic diseases. J Pediatr Hematol Oncol. 2009;31:505–8.
Khan RB, Sadighi ZS, Zabrowski J, Gajjar A, Jeha S. Imaging patterns and outcome of posterior reversible encephalopathy syndrome during childhood cancer treatment. Pediatr Blood Cancer. 2016;63:523–6.
Kim SJ, Im SA, Lee JW, et al. Predisposing factors of posterior reversible encephalopathy syndrome in acute childhood leukemia. Pediatr Neurol. 2012;47:436–42.
Panis B, Vlaar AM, van Well GT, et al. Posterior reversible encephalopathy syndrome in paediatric leukaemia. Eur J Paediatr Neurol. 2010;14:539–45.
Tang JH, Tian JM, Sheng M, et al. Study of posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia after induction chemotherapy. J Child Neurol. 2016;31:279–84.
Bartynski WS, Boardman JF, Zeigler ZR, Shadduck RK, Lister J. Posterior reversible encephalopathy syndrome in infection, sepsis, and shock. AJNR Am J Neuroradiol. 2006;27:2179–90.
Habetz K, Ramakrishnaiah R, Raina SK, Fitzgerald RT, Hinduja A. Posterior reversible encephalopathy syndrome: a comparative study of pediatric versus adult patients. Pediatr Neurol. 2016;65:45–51.
Raj S, Overby P, Erdfarb A, Ushay HM. Posterior reversible encephalopathy syndrome: incidence and associated factors in a pediatric critical care population. Pediatr Neurol. 2013;49:335–9.
Slater A, Shann F, Pearson G. Paediatric Index of Mortality Study G. PIM2: a revised version of the Paediatric Index of Mortality. Intensive Care Med. 2003;29:278–85.
National High Blood Pressure Education Program Working Group on High Blood Pressure in C, Adolescents. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics. 2004;114:555–76.
Fiser DH. Assessing the outcome of pediatric intensive care. J Pediatr. 1992;121:68–74.
Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. 2015;14:914–25.
Kastrup O, Gerwig M, Frings M, Diener HC. Posterior reversible encephalopathy syndrome (PRES): electroencephalographic findings and seizure patterns. J Neurol. 2012;259:1383–9.
Lee VH, Wijdicks EF, Manno EM, Rabinstein AA. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol. 2008;65:205–10.
Hobson EV, Craven I, Blank SC. Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness. Perit Dial Int. 2012;32:590–4.
Burnett MM, Hess CP, Roberts JP, Bass NM, Douglas VC, Josephson SA. Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors. Clin Neurol Neurosurg. 2010;112:886–91.
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Fisler, G., Monty, M.A., Kohn, N. et al. Characteristics and Outcomes of Critically Ill Pediatric Patients with Posterior Reversible Encephalopathy Syndrome. Neurocrit Care 32, 145–151 (2020). https://doi.org/10.1007/s12028-019-00720-9
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