{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T05:48:22Z","timestamp":1782107302455,"version":"3.54.5"},"reference-count":100,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,6,21]],"date-time":"2026-06-21T00:00:00Z","timestamp":1782000000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,21]],"date-time":"2026-06-21T00:00:00Z","timestamp":1782000000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Inborn errors of immunity (IEIs), an expanding group of monogenic disorders with diverse clinical manifestations, are increasingly recognized to include neuroinflammatory disease. Examples of diseases included under this umbrella are Aicardi\u2013Gouti\u00e8res syndrome, deficiency of adenosine deaminase 2, familial haemophagocytic lymphohistiocytosis, neonatal\u2010onset multisystem inflammatory disease, and acute necrotizing encephalopathy, among others. Children with IEIs may develop encephalopathy, seizures, focal neurological deficits, aseptic meningitis, inflammatory lesions on magnetic resonance imaging, or other central or peripheral nervous system manifestations. While systemic features of autoinflammation or autoimmunity are often present and provide important clues that an underlying IEI may be present, neuroinflammation may be the presenting or sole manifestation in some children. Early recognition of neuroinflammatory presentations of IEIs is critical to prompt immunological and genetic investigations, enabling diagnosis and timely initiation of appropriate immunotherapies, and reducing the risk of long\u2010term neurological outcomes. This review highlights paediatric\u2010onset neuroinflammatory phenotypes associated with IEIs and provides practical frameworks for their recognition and investigation in clinical practice.<\/jats:p>","DOI":"10.1111\/dmcn.70365","type":"journal-article","created":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T05:17:24Z","timestamp":1782105444000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Inborn errors of immunity in children with neuroinflammation"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6704-8402","authenticated-orcid":false,"given":"Eppie M","family":"Yiu","sequence":"first","affiliation":[{"name":"Department of Neurology Royal Children's Hospital Melbourne  Melbourne VIC Australia"},{"name":"Neuroscience Research Group Murdoch Children's Research Institute  Melbourne VIC Australia"},{"name":"Department of Paediatrics The University of Melbourne  Melbourne VIC Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4270-7015","authenticated-orcid":false,"given":"Dilan","family":"Dissanayake","sequence":"additional","affiliation":[{"name":"Departments of Paediatrics and Immunology University of Toronto  Toronto ON Canada"},{"name":"Division of Rheumatology The Hospital for Sick Children  Toronto ON Canada"},{"name":"Cell and Systems Biology Program SickKids Research Institute  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3453-0515","authenticated-orcid":false,"given":"Jessie","family":"Cunningham","sequence":"additional","affiliation":[{"name":"Health Sciences Library The Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7429-9012","authenticated-orcid":false,"given":"Helen","family":"Branson","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging and Interventional Radiology The Hospital for Sick Children  Toronto ON Canada"},{"name":"Department of Medical Imaging University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3470-5725","authenticated-orcid":false,"given":"Ori","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology and Allergy Hospital for Sick Children  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5393-7417","authenticated-orcid":false,"given":"E Ann","family":"Yeh","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Faculty of Medicine University of Toronto  Toronto ON Canada"},{"name":"Division of Neurology, Department of Paediatrics The Hospital for Sick Children  Toronto ON Canada"},{"name":"Division of Neuroscience and Mental Health SickKids Research Institute  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,21]]},"reference":[{"issue":"1","key":"e_1_2_10_2_1","article-title":"Human inborn errors of immunity: 2024 update on the classification from the International Union of Immunological Societies Expert Committee","volume":"1","author":"Poli MC","year":"2025","journal-title":"J Hum Immun."},{"issue":"3","key":"e_1_2_10_3_1","first-page":"76","article-title":"Inborn errors of immunity (primary immunodeficiencies). 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