{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T14:32:52Z","timestamp":1778509972118,"version":"3.51.4"},"reference-count":86,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2023,9,21]],"date-time":"2023-09-21T00:00:00Z","timestamp":1695254400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Autoinflammatory disorders encompass a wide range of conditions with systemic and neurological symptoms, which can be acquired or inherited. These diseases are characterized by an abnormal response of the innate immune system, leading to an excessive inflammatory reaction. On the other hand, autoimmune diseases result from dysregulation of the adaptive immune response. Disease flares are characterized by systemic inflammation affecting the skin, muscles, joints, serosa, and eyes, accompanied by unexplained fever and elevated acute phase reactants. Autoinflammatory syndromes can present with various neurological manifestations, such as aseptic meningitis, meningoencephalitis, sensorineural hearing loss, and others. Early recognition of these manifestations by general neurologists can have a significant impact on the prognosis of patients. Timely and targeted therapy can prevent long-term disability by reducing chronic inflammation. This review provides an overview of recently reported neuroinflammatory phenotypes, with a specific focus on genetic factors, clinical manifestations, and treatment options. General neurologists should have a good understanding of these important diseases.<\/jats:p>","DOI":"10.3390\/brainsci13091351","type":"journal-article","created":{"date-parts":[[2023,9,21]],"date-time":"2023-09-21T04:53:05Z","timestamp":1695271985000},"page":"1351","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["What General Neurologists Should Know about Autoinflammatory Syndromes?"],"prefix":"10.3390","volume":"13","author":[{"given":"Marianna Pinheiro Moraes","family":"de Moraes","sequence":"first","affiliation":[{"name":"Department of Neurology, Universidade Federal de S\u00e3o Paulo, S\u00e3o Paulo 04039-002, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2638-0086","authenticated-orcid":false,"given":"Renan Rodrigues Neves Ribeiro","family":"do Nascimento","sequence":"additional","affiliation":[{"name":"Departament of Rheumatology, Universidade Federal de S\u00e3o Paulo, S\u00e3o Paulo 04039-050, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3813-5993","authenticated-orcid":false,"given":"Fabiano Ferreira","family":"Abrantes","sequence":"additional","affiliation":[{"name":"Department of Neurology, Universidade Federal de S\u00e3o Paulo, S\u00e3o Paulo 04039-002, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 Luiz","family":"Pedroso","sequence":"additional","affiliation":[{"name":"Department of Neurology, Universidade Federal de S\u00e3o Paulo, S\u00e3o Paulo 04039-002, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1203-8287","authenticated-orcid":false,"given":"Sandro F\u00e9lix","family":"Perazzio","sequence":"additional","affiliation":[{"name":"Departament of Rheumatology, Universidade Federal de S\u00e3o Paulo, S\u00e3o Paulo 04039-050, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Orlando Graziani Povoas","family":"Barsottini","sequence":"additional","affiliation":[{"name":"Department of Neurology, Universidade Federal de S\u00e3o Paulo, S\u00e3o Paulo 04039-002, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,9,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1111\/j.1365-2249.2011.04535.x","article-title":"Immunology in clinic review series; focus on autoinflammatory diseases: Role of inflammasomes in autoinflammatory syndromes","volume":"167","author":"Ozkurede","year":"2012","journal-title":"Clin. 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