{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:09:42Z","timestamp":1753891782880,"version":"3.41.2"},"reference-count":13,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,4,18]],"date-time":"2024-04-18T00:00:00Z","timestamp":1713398400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p>Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is characterized by optic neuritis and meningoencephalomyelitis. We report the case of a 55-year-old man, otherwise healthy, who presented with isolated headaches for three months, without other features of meningoencephalitis or myelitis. His neurological examination and fundoscopy were unremarkable. Gadolinium-enhanced brain MRI demonstrated increased T2 hyperintensity within the right sub-lenticular basal ganglia, with additional leptomeningeal enhancement along the bilateral perisylvian regions and mesial temporal lobes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, elevated protein, matching oligoclonal bands, and a negative infectious and cytological workup. Cell-based assays for anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, autoimmune encephalitis panel, and vasculitis workup were all negative, except for CSF positivity for GFAP \u03b1 antibody. Oncological screening, including CT of the chest, abdomen, pelvis, and scrotal US, was unremarkable. Immunotherapy with high-dose intravenous steroids for five days and subsequent single four-weekly doses resulted in the resolution of both clinical and radiographic features, with a maintained status 24\u2009months after onset. This case highlights isolated headache and basal ganglia, mesial temporal lobe involvement as a rare presentation of autoimmune GFAP astrocytopathy.<\/jats:p>","DOI":"10.3389\/fneur.2024.1366263","type":"journal-article","created":{"date-parts":[[2024,4,18]],"date-time":"2024-04-18T04:42:11Z","timestamp":1713415331000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy"],"prefix":"10.3389","volume":"15","author":[{"given":"Eslam","family":"Shosha","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colleen","family":"Connolly","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adrian","family":"Budhram","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,4,18]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1297","DOI":"10.1001\/jamaneurol.2016.2549","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis","volume":"73","author":"Fang","year":"2016","journal-title":"JAMA Neurol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1016\/j.jneuroim.2018.04.016","article-title":"Autoimmune GFAP astrocytopathy: prospective evaluation of 90 patients in 1 year","volume":"321","author":"Dubey","year":"2018","journal-title":"J Neuroimmunol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1186\/s12883-020-02021-7","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy manifesting as subacute meningoencephalitis with descending myelitis: a case report","volume":"20","author":"Wang","year":"2020","journal-title":"BMC Neurol"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1111\/ene.13527","article-title":"A path to understanding autoimmune GFAP astrocytopathy","volume":"25","author":"Zekeridou","year":"2018","journal-title":"Eur J Neurol"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1136\/jnnp-2017-316583","article-title":"Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients","volume":"89","author":"Iorio","year":"2018","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1002\/ana.24881","article-title":"Glial fibrillary acidic protein immunoglobulin gas biomarker of autoimmune astrocytopathy: analysis of 102 patients","volume":"81","author":"Flanagan","year":"2017","journal-title":"Ann Neurol"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"3031","DOI":"10.2169\/internalmedicine.6457-20","article-title":"Self-remitting elevation of adenosine deaminase levels in the cerebrospinal fluid with autoimmune glial fibrillary acidic protein Astrocytopathy: a case report and review of the literature","volume":"60","author":"Nakamura","year":"2021","journal-title":"Intern Med"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"hcae 007","DOI":"10.1093\/qjmed\/hcae007","article-title":"Headache and optic disc oedema revealing autoimmune GFAP-astrocytopathy","author":"Ng","year":"2024","journal-title":"QJM"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"e653","DOI":"10.1212\/WNL.0000000000013087","article-title":"Glial fibrillary acidic protein autoimmunity: a French cohort study","volume":"98","author":"Gravier-Dumonceau","year":"2022","journal-title":"Neurology"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1111\/ene.13531","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study","volume":"25","author":"Long","year":"2018","journal-title":"Eur J Neurol"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1016\/j.msard.2018.02.020","article-title":"Autoimmune GFAP astrocytopathy after viral encephalitis: a case report","volume":"21","author":"Li","year":"2018","journal-title":"Mult Scler Relat Disord"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1016\/j.jneuroim.2019.04.004","article-title":"Clinical characteristics of autoimmune GFAP astrocytopathy","volume":"332","author":"Takekoshi","year":"2019","journal-title":"J Neuroimmunol"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1159\/000479948","article-title":"Treatment of autoimmune glial fibrillary acidic protein astrocytopathy: follow-up in 7 cases","volume":"24","author":"Yang","year":"2017","journal-title":"Neuroimmunomodulation"}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2024.1366263\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,18]],"date-time":"2024-04-18T04:42:13Z","timestamp":1713415333000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2024.1366263\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,18]]},"references-count":13,"alternative-id":["10.3389\/fneur.2024.1366263"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2024.1366263","relation":{},"ISSN":["1664-2295"],"issn-type":[{"type":"electronic","value":"1664-2295"}],"subject":[],"published":{"date-parts":[[2024,4,18]]},"article-number":"1366263"}}