Lethal Pediatric Cerebral Vasculitis Triggered by Severe Acute Respiratory Syndrome Coronavirus 2 - 17/01/22
Abstract |
Background |
We report the clinical, radiological, laboratory, and neuropathological findings in support of the first diagnosis of lethal, small-vessel cerebral vasculitis triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a pediatric patient.
Patient Description |
A previously healthy, eight-year-old Hispanic girl presented with subacute left-sided weakness two weeks after a mild febrile illness. SARS-CoV-2 nasopharyngeal swab was positive. Magnetic resonance imaging revealed an enhancing right frontal lobe lesion with significant vasogenic edema. Two brain biopsies of the lesion showed perivascular and intraluminal lymphohistiocytic inflammatory infiltrate consistent with vasculitis. Despite extensive treatment with immunomodulatory therapies targeting primary angiitis of the central nervous system, she experienced neurological decline and died 93 days after presentation. SARS-CoV-2 testing revealed positive serum IgG and positive cerebrospinal fluid IgM. Comprehensive infectious, rheumatologic, hematologic/oncologic, and genetic evaluation did not identify an alternative etiology. Postmortem brain autopsy remained consistent with vasculitis.
Conclusion |
This is the first pediatric presentation to suggest that SARS-CoV-2 can lead to a fatal, postinfectious, inflammatory small-vessel cerebral vasculitis. Our patient uniquely included supportive cerebrospinal fluid and postmortem tissue analysis. While most children recover from the neurological complications of SARS-CoV-2, we emphasize the potential mortality in a child with no risk factors for severe disease.
Le texte complet de cet article est disponible en PDF.Keywords : SARS-CoV-2, COVID-19, Pediatrics, Stroke, Cerebral vasculitis
Plan
Authors' Contributions: All authors contributed to the conception and design of the study, data acquisition and analysis, and drafting the article. Final approval was obtained from all authors before submission. |
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Declarations of Interests: None. |
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Funding/support: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. |
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Previous presentation of the information in this manuscript: Our patient was included in very limited detail in the study “Neurologic Involvement in Children and Adolescents Hospitalized in the United States for COVID-19 or Multisystem Inflammatory Syndrome,” published in JAMA Neurology in March 2021. In that study's text our patient is the fourth stroke patient who died, and her course is summarized in one sentence within the body of the manuscript. In supplemental e-Table 4 she is presented as Case 3. The authors of that study had limited access to data and no access to imaging on our patient, and their manuscript was published before our patient's postmortem autopsy was performed and the CSF SARS-CoV-2 IgM result was known. |
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Disclosures: Authors have no potential, perceived, or real conflicts of interest to report relative to this study. This research was not funded by any sponsor. Study design, collection, analysis and interpretation of data, and all subsequent writing as well as decisions on manuscript submission were made at authors' discretion without undue influence. Drafting and revision of manuscript was performed by all study authors without honorarium, grant, or other form of payment. |
Vol 127
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