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Characteristics and Outcomes of Critically Ill Pediatric Patients with Posterior Reversible Encephalopathy Syndrome

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Abstract

Background/Objective

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.

Methods

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.


Results

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).

Conclusions

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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Abbreviations

AED:

Antiepileptic drug

AMS:

Altered mental status

DBP:

Diastolic blood pressure

LEV:

Levetiracetam

MAP:

Mean arterial pressure

MRI:

Magnetic resonance imaging

MRV:

Magnetic resonance venogram

PICU:

Pediatric intensive care unit

PIM-2:

Pediatric Index of Mortality-2

PRES:

Posterior reversible encephalopathy syndrome

SBP:

Systolic blood pressure

SLE:

Systemic lupus erythematous

PCPC:

Pediatric cerebral performance category

POPC:

Pediatric overall performance category

References

  1. Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med. 1996;334:494–500.

    Article  CAS  Google Scholar 

  2. Stevens CJ, Heran MK. The many faces of posterior reversible encephalopathy syndrome. Br J Radiol. 2012;85:1566–75.

    Article  CAS  Google Scholar 

  3. 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.

    Article  Google Scholar 

  4. 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.

    Article  Google Scholar 

  5. 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.

    Article  Google Scholar 

  6. 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.

    Article  Google Scholar 

  7. 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.

    Article  Google Scholar 

  8. Yamada A, Ueda N. Age and gender may affect posterior reversible encephalopathy syndrome in renal disease. Pediatr Nephrol. 2012;27:277–83.

    Article  Google Scholar 

  9. 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.

    PubMed  Google Scholar 

  10. Onder AM, Lopez R, Teomete U, et al. Posterior reversible encephalopathy syndrome in the pediatric renal population. Pediatr Nephrol. 2007;22:1921–9.

    Article  Google Scholar 

  11. 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.

    Article  Google Scholar 

  12. Hagemann G, Ugur T, Witte OW, Fitzek C. Recurrent posterior reversible encephalopathy syndrome (PRES). J Hum Hypertens. 2004;18:287–9.

    Article  CAS  Google Scholar 

  13. 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.

    Article  Google Scholar 

  14. 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.

    Article  CAS  Google Scholar 

  15. 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.

    Article  Google Scholar 

  16. Panis B, Vlaar AM, van Well GT, et al. Posterior reversible encephalopathy syndrome in paediatric leukaemia. Eur J Paediatr Neurol. 2010;14:539–45.

    Article  Google Scholar 

  17. 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.

    Article  Google Scholar 

  18. 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.

    CAS  PubMed  Google Scholar 

  19. 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.

    Article  Google Scholar 

  20. 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.

    Article  Google Scholar 

  21. 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.

    Article  Google Scholar 

  22. 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.

    Article  Google Scholar 

  23. Fiser DH. Assessing the outcome of pediatric intensive care. J Pediatr. 1992;121:68–74.

    Article  CAS  Google Scholar 

  24. Fugate JE, Rabinstein AA. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol. 2015;14:914–25.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  26. Lee VH, Wijdicks EF, Manno EM, Rabinstein AA. Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol. 2008;65:205–10.

    PubMed  Google Scholar 

  27. Hobson EV, Craven I, Blank SC. Posterior reversible encephalopathy syndrome: a truly treatable neurologic illness. Perit Dial Int. 2012;32:590–4.

    Article  Google Scholar 

  28. 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.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Graduate Medical Education Office, Steven and Alexandra Cohen Children’s Medical Center of New York, 269-01 76th Ave New, Hyde Park, NY, 11040, USA

    Grace Fisler

  2. Department of Pediatrics, Hasbro Children’s Hospital, 593 Eddy Street, Providence, RI, 02903, USA

    Mikhaela A. Monty

  3. Biostatistics Unit, Feinstein Institute for Medical Research, 125 Community Drive, Great Neck, NY, 11021, USA

    Nina Kohn

  4. Department of Pediatric Radiology, Steven and Alexandra Cohen Children’s Medical Center of New York, 269-01 76th Ave, New Hyde Park, NY, 11040, USA

    Peter Assaad

  5. Pediatric Critical Care Medicine, Steven and Alexandra Cohen Children’s Medical Center of New York, 269-01 76th Ave, New Hyde Park, NY, 11040, USA

    Randi Trope & Aaron Kessel

Authors
  1. Grace Fisler
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  2. Mikhaela A. Monty
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  3. Nina Kohn
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  4. Peter Assaad
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  5. Randi Trope
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  6. Aaron Kessel
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Contributions

GF contributed to study design, data collection, and manuscript preparation; MAM involved in data collection and manuscript preparation; NK took part in study design and statistical analysis; PA contributed to MRI review and manuscript preparation; RT involved in study design and manuscript preparation; and AK took part in principle investigator, study design, and manuscript preparation.

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Correspondence to Grace Fisler.

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All authors have no conflicts of interest.

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This study was approved by the Northwell Health System Institutional Review Board.

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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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  • DOI: https://doi.org/10.1007/s12028-019-00720-9

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