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SARS-CoV-2-Related Acute Necrotizing Encephalopathy of Childhood With Good Response to Tocilizumab in an Adolescent - 12/01/23

Doi : 10.1016/j.pediatrneurol.2022.11.010 
Jasmine H.Y. Ho, MB BChir, MA Hons (Cantab), MMed (Paeds), MRCPCH a, Cheryl Y.M. Lee, MBBS, MMed (Paeds), MRCPCH a, Yee Keow Chiong, MBBS, MMed (Paeds), MRCPCH a, Rie Aoyama, MB BCh, BaO (NUI, RCSI) LRCP&SI, MMed (Paeds), MRCPCH a, Li Jia Fan, MBBS, MMed (Paeds), MRCPCH a, Amos H.S. Tan, MBBS, FRCR, MMed (Diagnostic Radiology) b, Velda X. Han, MBBS, MMed (Paeds), MRCPCH a, c,
a Khoo Teck Puat - National University Children's Medical Institute, National University Hospital, Singapore, Singapore 
b Department of Diagnostic Imaging, National University Hospital, Singapore, Singapore 
c Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore 

Communications should be addressed to: Dr. Han; Department of Paediatrics; Khoo Teck Puat - National University Children's Medical Institute; National University Hospital of Singapore; 5 Lower Kent Ridge Road; Singapore 119074, Singapore.Department of PaediatricsKhoo Teck Puat - National University Children's Medical InstituteNational University Hospital of Singapore5 Lower Kent Ridge RoadSingapore119074Singapore

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Abstract

Background

Acute necrotizing encephalopathy of childhood (ANEC) is a rare parainfectious neurological disorder. ANEC is associated with a high mortality rate and poor neurological outcomes. ANEC is postulated to arise from immune-mediated or metabolic processes driven by viral infections. Although there have been some case reports of acute necrotizing encephalopathy with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) coinfection in adults, paediatric cases are rare.

Methods

A single case report of SARS-CoV-2-related ANEC in an 11-year-old boy is presented through retrospective chart review. Literature search was performed using PubMed, Embase, Cochrane database, and Google Scholar to compare and analyze similar cases of parainfectious immune-mediated encephalopathies related to SARS-CoV-2 in children.

Results

An 11-year-old boy with acute SARS-CoV-2 infection presented with ophthalmoplegia, ataxia, and aphasia. Neuroimaging findings demonstrated significant swelling and signal changes in bilateral thalami, brainstem, and cerebellar hemispheres, consistent with ANEC. His high ANEC Severity Score indicated poor neurological prognosis. Treatment with a combination of early steroid therapy, intravenous immunoglobulin therapy, and targeted interleukin 6 (IL-6) blockade yielded good neurological improvements. Literature search identified 19 parainfectious immune-mediated neurological disorders related to SARS-CoV-2 in children. The only other pediatric ANEC case identified was postinfectious and thus not included.

Conclusions

This is the first report of a pediatric case of SARS-CoV-2-related ANEC, which responded well to early immunotherapy, including IL-6 blockade. Early immunotherapy with IL-6 blockade can be considered as an adjunct in managing severe ANEC.

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Keywords : COVID-19, SARS-CoV-2, Acute necrotizing encephalopathy, Pediatrics, Pediatric neurology, Tocilizumab


Plan


 Conflicts of Interest: The authors have no relevant financial or nonfinancial interests to disclose.
 Author Contributions: The first draft of the manuscript was written by Dr. Cheryl Y.M. Lee, Dr. Jasmine H.Y. Ho, and Dr. Chiong Y.K. All authors read, critically revised, and approved the final manuscript.
 Funding Statement: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
 Data Availability Statement: Not applicable as no datasets were generated or analyzed in this study.
 Ethics Approval and Consent to Participate: Assent was obtained from the patient, and informed consent was obtained from the patient's parent.
 Consent for Publication: Consent for publication was obtained from the patient's parent.


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Vol 139

P. 65-69 - février 2023 Retour au numéro
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