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

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

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Author information

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.

Correspondence to Grace Fisler.

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All authors have no conflicts of interest.

Ethical Approval/Informed Consent

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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Keywords

  • Posterior reversible encephalopathy syndrome
  • PRES
  • Hypertension
  • Seizures
  • Immunosuppression