COVID-19-associated Acute Disseminated Encephalomyelitis-like Disease in 2 Children

Abstract

Background: The coronavirus disease 2019 pandemic was caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Although the predominant clinical presentation is a respiratory disease, neurologic manifestations are being recognized increasingly.

Case report: We report 2 children 9 years of age who developed acute disseminated encephalomyelitis-like disease associated with SARS-CoV-2. Seizures and encephalopathy were the main central nervous system symptoms. The cerebrospinal fluid analysis performed within the first week of disease onset showed elevated protein in both children with normal cell count and no evidence of infection including negative SARS-CoV-2 by antibody and polymerase chain reaction. Brain magnetic resonance imaging revealed T2A, fluid-attenuated inversion recovery cortical and subcortical hyperintensity without restricted diffusion consistent with acute disseminated encephalomyelitis-like disease. They received methylprednisolone followed by therapeutic plasma exchange. One of them showed complete clinical improvement and resolution in magnetic resonance imaging findings. The other developed laminar necrosis in brain magnetic resonance imaging and showed significant clinical improvement after therapeutic plasma exchange. He was positive for positive SARS-CoV-2 antibody in cerebrospinal fluid on day 55 of admission. They were both positive for SARS-CoV-2 antibodies in serum after 2 weeks.

Conclusions: Our two cases highlight the occurrence of acute disseminated encephalomyelitis-like disease as a postinfectious/immune-mediated complication of SARS-CoV-2 infection.

FIGURE 1.

Brain MRI of a 9-year-old boy at presentation. In the fluid-attenuated inversion recovery sequences, pathologic signal changes were observed in cortical and subcortical areas in bilateral frontoparietal regions (A–C). These lesions showed contrast enhancement after contrast enhancement (D–F) and marked diffusion restriction in apparent diffusion coefficient mapping in diffusion-weighted sequences (G and H).

FIGURE 2.

Brain MRI of a 9-year-old boy on day 9. The lesions continued in fluid-attenuated inversion recovery sequences (A and B) and cortical linear hyperintensities compatible with laminar necrosis occurred in T1A sequences (C). It was found that the restriction disappeared in the diffusion-weighted sequences (D) and contrast enhancement continued in the frontoparietal regions after contrast agent administration (E and F) and necrotic areas began to occur in these regions.

FIGURE 3.

Brain MRI of a 9-year-old boy on day 20. Prominent areas of necrosis in both frontoparietal regions (A–F).

FIGURE 4.

Brain MRI of a 9-year-old boy on day 33. Pathologic signal changes, contrast enhancements, and laminar necrosis continued in both frontoparietal regions, areas of cystic necrosis became prominent, and atrophy occurred in the brain (A–H).

FIGURE 5.

Brain MRI of a 9-year-old girl at presentation. In the fluid-attenuated inversion recovery (FLAIR) sequences, pathologic signal increases were observed in the bilateral temporal region in the hippocampal areas, bilateral thalamus, putamen and deep white matter. Diffusion restriction was not observed in these lesions in diffusion-weighted sequences. These lesions were evaluated as acute disseminated encephalomyelitis–like diseases (A–D). MRI 12 days later (E–H) showed the lesions previously detected in FLAIR sequences disappeared but new lesions developed in the right temporal lobe inferior, bilateral occipital localization, cortical and subcortical areas extending toward the corona radiata and showing no diffusion restriction. These newly formed lesions were considered as posterior reversible encephalopathy syndrome.