Preprint Review Version 1 Preserved in Portico This version is not peer-reviewed

What General Neurologists Should Know about Autoinflammatory Syndromes?

Version 1 : Received: 10 August 2023 / Approved: 11 August 2023 / Online: 14 August 2023 (02:44:49 CEST)

A peer-reviewed article of this Preprint also exists.

de Moraes, M.P.M.; do Nascimento, R.R.N.R.; Abrantes, F.F.; Pedroso, J.L.; Perazzio, S.F.; Barsottini, O.G.P. What General Neurologists Should Know about Autoinflammatory Syndromes? Brain Sci. 2023, 13, 1351. de Moraes, M.P.M.; do Nascimento, R.R.N.R.; Abrantes, F.F.; Pedroso, J.L.; Perazzio, S.F.; Barsottini, O.G.P. What General Neurologists Should Know about Autoinflammatory Syndromes? Brain Sci. 2023, 13, 1351.

Abstract

Autoinflammatory disorders encompass a wide range of conditions with systemic and neuro-logical 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 reac-tion. On the other hand, autoimmune diseases result from dysregulation of the adaptive im-mune 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 neuroinflam-matory phenotypes, with a specific focus on genetic factors, clinical manifestations, and treatment options. General neurologists should have a good understanding of these important diseases.

Keywords

Autoinflammatory Disease; Hereditary Periodic Fever Syndromes; Hereditary Recurrent Fever; Hereditary Autoinflammatory Disease

Subject

Medicine and Pharmacology, Neuroscience and Neurology

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