Pediatr Emerg Med J.  2023 Oct;10(4):142-148. 10.22470/pemj.2023.00724.

Coronavirus disease 2019-associated acute necrotizing encephalopathy in a 9-year-old boy

Affiliations
  • 1Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea
  • 2Division of Pediatric Critical Care Medicine, Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea

Abstract

Coronavirus disease 2019 (COVID-19) is associated with a variety of neurologic manifestations. Acute necrotizing encephalopathy (ANE) is a rare, life-threatening complication characterized by rapid deterioration of neurologic status following viral infection, such as influenza or human herpesvirus 6. Since the COVID-19 pandemic, a rise in ANE cases associated with the infectious disease has been reported in adult patients. We present a case of COVID-19-associated ANE in a 9-year-old boy. The patient experienced 3 days of fever and mild respiratory symptoms, followed by lethargy. Magnetic resonance imaging on day 4 showed hyperintensity in the bilateral thalami, midbrain, pons, hypothalamus, and cerebellum, along with some areas of hemorrhage. From the imaging findings, ANE was strongly suspected, leading to the initiation treatment involving a 5-day course of remdesivir and multiple immunomodulator therapies, including high-dose corticosteroids, intravenous immunoglobulin, tocilizumab, and 10 cycles of therapeutic plasma exchange. Subsequently, the patient gradually improved, experiencing only minor neurological sequelae and showing favorable radiologic improvement. In COVID-19-infected patients presenting neurologic symptoms, it is crucial to promptly suspect and investigate unexplained encephalopathy using neuroimaging. Early administration of immunomodulator therapy is vital for the diagnosis and optimizing clinical outcomes.

Keyword

Brain Diseases; COVID-19; Immunologic Factors; Plasma Exchange; SARS-CoV-2

Figure

  • Fig. 1. Computed tomography scans of the patient on day 1, showing hypodense lesions (arrows) in both thalami (A) and pons (B).

  • Fig. 2. Magnetic resonance imaging on day 3. The fluid-attenuated inversion recovery images show hyperintense lesions of both thalami (arrows, A), midbrain (image not shown), pons (arrows, B), hypothalamus (image not shown), and cerebellum (arrowhead, B). The diffusion-weighted images show restrictive lesions in both thalami (arrows, C), pons (arrows, D), and cerebellum (arrowhead, D). The susceptibility-weighted images show hypointense hemorrhagic foci in both thalami (arrows, E), pons (arrows F), and hypothalamus (image not shown).

  • Fig. 3. Follow-up magnetic resonance imaging on day 24. The fluid-attenuated inversion recovery (A) and T2-weighted (B) images show nearly eliminated hyperintensities involving both thalami (arrows, A), as well as the midbrain (image not shown), pons (arrows, B), hypothalamus, and left cerebellum (images not shown). The susceptibility-weighted images show multifocal hemorrhagic foci in the pons (arrows, C), midbrain (image not shown), and both thalami (arrows, D).


Reference

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