PC1178: Stop the PRES: A New MIS-C Complication

Introduction: Multisystem inflammatory syndrome in children (MIS-C) and its management predispose pediatric patients to a neurologic complication called posterior reversible encephalopathy syndrome (PRES). This is the first reported case of PRES in MIS-C. PRES has only been identified in one pediatric patient with acute SARS-CoV-2 infection (Korkmazer et al., 2021).

Case Description: An 8-year-old male with history of SARS-CoV-2 infection four weeks prior initially presented with fever for 4 days, headache, abdominal pain, vomiting, and diarrhea. He was diagnosed with MIS-C with supporting lab and echocardiogram findings. He was started on methylprednisolone 2 mg/kg/day (70 mg/day), which was later increased to 4 mg/kg/day (140 mg/day) when his cardiac markers and echocardiogram did not improve despite also receiving IVIG. While receiving this higher dose of steroids for 2.5 days, his blood pressure progressively increased (Figure 1).

On hospital day 8, the patient was found to be unresponsive with eye deviation and flaccid posturing. His last measured blood pressure was 155/73. Acute management in the PICU included: intubation with minimal sedation for hypoxia and altered mental status, Nicardipine drip for sustained hypertension, and levetiracetam loading dose for clinical concern for seizures. His non-contrast head CT and continuous video electroencephalogram were both normal. The next day, MRI of his brain showed bilateral paramedian parietal subcortical fluid-attenuated inversion recovery (FLAIR) signal hyperintensity (Figure 2), leading to his diagnosis of PRES.

Discussion: PRES presents with acute onset of neurologic symptoms typically in the setting of hypertension and characteristic findings on MRI. Neurologic symptoms include seizures, altered mental status, headaches, visual disturbances, nausea or vomiting, or focal neural deficits. Hypertension has been reported in 70-80% of pediatric patients. According to one study, the mean blood pressure at the onset of PRES was 140/85. This corresponds to the case patient’s average blood pressure of 147/87 in the 24 hours prior to his transfer to the PICU. MRI reveals hyperintensities on T2 weighted MRI and FLAIR imaging in the cortical and subcortical regions of the parietal and occipital lobes. Early diagnosis and initiation of treatment are essential to a favorable prognosis (Chen, 2020).

Known risk factors of PRES include IVIG and high dose corticosteroids, which are considered the mainstay of treatment for MIS-C. The most recent MIS-C guidelines recommend 1-2 mg/kg/day of methylprednisolone for the initial treatment and up to 10-30 mg/kg/day for refractory disease (Henderson et al., 2022). It should be noted that these immunomodulatory agents, especially at such high doses, increase the risk of developing PRES.

Conclusion: This case argues the need for aggressive blood pressure monitoring and management for patients being treated for MIS-C. It is essential that pediatricians do not neglect rising blood pressures in these patients and act early to prevent the onset of PRES.