{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T16:41:01Z","timestamp":1789663261064,"version":"4.0.1"},"reference-count":46,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T00:00:00Z","timestamp":1648512000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP21K07457"],"award-info":[{"award-number":["JP21K07457"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is a type of autoimmune corticosteroid-responsive meningoencephalitis that occurs with or without myelitis. Movement disorders have been reported in GFAP-A patients but have not been characterized. In this study, we examined the characteristics of movement disorders in GFAP-A patients. We retrospectively reviewed clinical data from 87 consecutive patients with GFAP-A attending Gifu University Hospital in Japan. We compared the demographics, clinical features, cerebrospinal fluid characteristics, and neuroimaging findings from patients with and without movement disorders. Seventy-four patients (85%) had movement disorders, including ataxia (49%), tremor (45%), myoclonus (37%), dyskinesia (2%), opsoclonus (2%), rigidity (2%), myokymia (1%), and choreoathetosis (1%). GFAP-A patients with movement disorders were significantly older than those without. Movement disorders are therefore common in GFAP-A patients, and the main types of movement disorders observed in this population were ataxia, tremor, and myoclonus. These abnormal movements can serve as clinical features that facilitate the early diagnosis of GFAP-A. Elderly GFAP-A patients are more likely to have movement disorder complications than younger patients.<\/jats:p>","DOI":"10.3390\/brainsci12040462","type":"journal-article","created":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T21:40:14Z","timestamp":1648590014000},"page":"462","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Characteristics of Movement Disorders in Patients with Autoimmune GFAP Astrocytopathy"],"prefix":"10.3390","volume":"12","author":[{"given":"Akio","family":"Kimura","sequence":"first","affiliation":[{"name":"Department of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Takekoshi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8788-9089","authenticated-orcid":false,"given":"Takayoshi","family":"Shimohata","sequence":"additional","affiliation":[{"name":"Department of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu 501-1194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,3,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","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":"ref_2","doi-asserted-by":"crossref","first-page":"298","DOI":"10.1002\/ana.24881","article-title":"Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients","volume":"81","author":"Flanagan","year":"2017","journal-title":"Ann. Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","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":"ref_4","doi-asserted-by":"crossref","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":"ref_5","doi-asserted-by":"crossref","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":"ref_6","first-page":"e653","article-title":"Glial fibrillary acidic protein autoimmunity: A French cohort study","volume":"98","author":"Dumonceau","year":"2022","journal-title":"Neurology"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1136\/practneurol-2017-001863","article-title":"Glial fibrillary acidic protein antibody-positive meningoencephalomyelitis","volume":"18","author":"Zarkali","year":"2018","journal-title":"Pract. Neurol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.jneuroim.2019.04.004","article-title":"Clinical characteristics of autoimmune GFAP astrocytopathy","volume":"332","author":"Kimura","year":"2019","journal-title":"J. Neuroimmunol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.jpag.2017.12.009","article-title":"Ovarian teratoma associated with coexisting anti-N-methyl-D-aspartate receptor and glial fibrillary acidic protein autoimmune meningoencephalitis in an adolescent girl: A case report","volume":"31","author":"Martin","year":"2018","journal-title":"J. Pediatr. Adolesc. Gynecol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1159\/000488879","article-title":"Brain immunohistopathology in a patient with autoimmune glial fibrillary acidic protein astrocytopathy","volume":"25","author":"Shu","year":"2018","journal-title":"Neuroimmunomodulation"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2121","DOI":"10.1111\/ene.14778","article-title":"CD8+ T-cell predominance in autoimmune glial fibrillary acidic protein astrocytopathy","volume":"28","author":"Yuan","year":"2021","journal-title":"Eur. J. Neurol."},{"key":"ref_12","doi-asserted-by":"crossref","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"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1097\/WCO.0000000000000676","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy","volume":"32","author":"Kunchok","year":"2019","journal-title":"Curr. Opin. Neurol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"101900","DOI":"10.1016\/j.msard.2019.101900","article-title":"A case of GFAP-astroglial autoimmunity presenting with reversible Parkinsonism","volume":"39","author":"Tomczak","year":"2019","journal-title":"Mult. Scler. Relat. Disord."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"e000142","DOI":"10.1136\/bmjno-2021-000142","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy presented as ataxia, myoclonus and bulbar syndrome: A case report and review of the literature","volume":"3","author":"Novo","year":"2021","journal-title":"BMJ Neurol. Open"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"17562864211062824","DOI":"10.1177\/17562864211062824","article-title":"Anti-GFAP-antibody positive postinfectious acute cerebellar ataxia and myoclonus after COVID-19: A case report","volume":"14","author":"Asan","year":"2021","journal-title":"Ther. Adv. Neurol. Disord."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"251","DOI":"10.3389\/fneur.2018.00251","article-title":"Overlapping autoimmune syndromes in patients with glial fibrillary acidic protein antibodies","volume":"9","author":"Yang","year":"2018","journal-title":"Front. Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.msard.2018.09.015","article-title":"Extreme delta brush and distinctive imaging in a pediatric patient with autoimmune GFAP astrocytopathy","volume":"26","author":"Theroux","year":"2018","journal-title":"Mult. Scler. Relat. Disord."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"e481","DOI":"10.1212\/NXI.0000000000000481","article-title":"GFAP\u03b1 IgG-associated encephalitis upon daclizumab treatment of MS","volume":"5","author":"Luessi","year":"2018","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1136\/jnnp-2018-318004","article-title":"Glial fibrillary acidic protein IgG related myelitis: Characterisation and comparison with aquaporin-4-IgG myelitis","volume":"90","author":"Sechi","year":"2019","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"100229","DOI":"10.1016\/j.ensci.2020.100229","article-title":"Abnormal evoked potentials in autoimmune glial fibrillary acidic protein astrocytopathy","volume":"18","author":"Tokimura","year":"2020","journal-title":"eNeurologicalSci."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1159\/000505730","article-title":"Overlapping clinical syndromes in patients with glial fibrillary acidic protein IgG","volume":"27","author":"Lin","year":"2020","journal-title":"Neuroimmunomodulation"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.ejpn.2020.03.002","article-title":"Mild encephalitis\/encephalopathy with reversible splenial lesion syndrome: An unusual presentation of anti-GFAP astrocytopathy","volume":"26","author":"Oger","year":"2020","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2999","DOI":"10.1007\/s10072-020-04454-x","article-title":"A treatable case of autoimmune GFAP astrocytopathy presenting chronic progressive impairment","volume":"41","author":"Natori","year":"2020","journal-title":"Neurol. Sci."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1007\/s00259-020-05001-8","article-title":"[18F]-FDG PET imaging in autoimmune GFAP meningoencephalomyelitis","volume":"48","author":"Rosales","year":"2021","journal-title":"Eur. J. Nucl. Med. Mol. Imaging"},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Xu, L., Xian, W., Li, J., Yao, X., and Long, Y. (2020). Purkinje cell (PC) antibody positivity in a patient with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Int. J. Neurosci., 1\u20139.","DOI":"10.1080\/00207454.2020.1860965"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1111\/ncn3.12441","article-title":"A case of autoimmune GFAP astrocytopathy with profound weight loss and increased uptake in the spinal cord on 18F-FDG PET","volume":"9","author":"Osakada","year":"2021","journal-title":"Neurol. Clin. Neurosci."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Wang, H., Chin, J.H., Fang, B.Y., Chen, X., Zhao, A.L., Ren, H.T., and Guan, H.Z. (2020). Autoimmune glial fibrillary acidic protein astrocytopathy manifesting as subacute meningoencephalitis with descending myelitis: A case report. BMC Neurol., 20.","DOI":"10.1186\/s12883-020-02021-7"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"626564","DOI":"10.3389\/fped.2020.626564","article-title":"Glial fibrillary acidic protein astrocytopathy in pediatric patients: A retrospective study","volume":"8","author":"Huang","year":"2021","journal-title":"Front. Pediatr."},{"key":"ref_30","doi-asserted-by":"crossref","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":"ref_31","doi-asserted-by":"crossref","first-page":"102933","DOI":"10.1016\/j.msard.2021.102933","article-title":"Failure of IVIG in steroid-responsive autoimmune glial fibrillary acidic protein astrocytopathy: A case report","volume":"51","author":"Heide","year":"2021","journal-title":"Mult. Scler. Relat. Disord."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"2256","DOI":"10.1007\/s00415-021-10484-y","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy: Case report of a treatable cause of rapidly progressive dementia","volume":"268","year":"2021","journal-title":"J. Neurol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"401","DOI":"10.5692\/clinicalneurol.cn-001575","article-title":"A case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy","volume":"61","author":"Adachi","year":"2021","journal-title":"Rinsho. Shinkeigaku"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1002\/ana.26195","article-title":"Magnetic resonance imaging of autoimmune GFAP astrocytopathy","volume":"90","author":"Tewkesbury","year":"2021","journal-title":"Ann. Neurol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"577695","DOI":"10.1016\/j.jneuroim.2021.577695","article-title":"Autoimmune GFAP astrocytopathy presenting with remarkable CNS hyperexcitability and oculogyric crises","volume":"359","author":"Equiza","year":"2021","journal-title":"J. Neuroimmunol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"577718","DOI":"10.1016\/j.jneuroim.2021.577718","article-title":"Clinical, neuroradiological, diagnostic and prognostic profile of autoimmune glial fibrillary acidic protein astrocytopathy: A pooled analysis of 324 cases from published data and a single-center retrospective study","volume":"360","author":"Xiao","year":"2021","journal-title":"J. Neuroimmunol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"e1081","DOI":"10.1212\/NXI.0000000000001081","article-title":"Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody","volume":"8","author":"Yamakawa","year":"2021","journal-title":"Neurol. Neuroimmunol. Neuroinflamm."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"577748","DOI":"10.1016\/j.jneuroim.2021.577748","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy resembling isolated central nervous system lymphomatoid granulomatosis","volume":"361","author":"Kimura","year":"2021","journal-title":"J. Neuroimmunol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1001\/jamaneurol.2021.4054","article-title":"Linear radial brainstem enhancement in autoimmune glial fibrillary acidic protein astrocytopathy","volume":"79","author":"Mirian","year":"2021","journal-title":"JAMA Neurol."},{"key":"ref_40","doi-asserted-by":"crossref","unstructured":"H\u00e9raud, C., Capet, N., Levraut, M., Hattenberger, R., Bourg, V., Thomas, P., Mondot, L., and Lebrun-Frenay, C. (2021). Glial fibrillary acidic protein (GFAP) astrocytopathy presenting as mild encephalopathy with reversible splenium lesion. Neurol. Ther., Online ahead of print.","DOI":"10.1007\/s40120-021-00302-y"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"761354","DOI":"10.3389\/fimmu.2021.761354","article-title":"Autoimmune glial fibrillary acidic protein astrocytopathy in children: A retrospective analysis of 35 cases","volume":"12","author":"Fang","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"3297","DOI":"10.1093\/brain\/awm266","article-title":"Neuropathological changes in essential tremor: 33 cases compared with 21 controls","volume":"130","author":"Louis","year":"2007","journal-title":"Brain"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1016\/S1474-4422(10)70090-9","article-title":"Essential tremor: Evolving clinicopathological concepts in an era of intensive post-mortem enquiry","volume":"9","author":"Louis","year":"2010","journal-title":"Lancet Neurol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1038\/s41572-021-00314-w","article-title":"Essential tremor","volume":"7","author":"Welton","year":"2021","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1097\/WCO.0b013e328347b307","article-title":"Movement disorders in paraneoplastic and autoimmune disease","volume":"24","author":"Panzer","year":"2011","journal-title":"Curr. Opin. Neurol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/S1474-4422(19)30244-3","article-title":"An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: Mechanisms and models","volume":"18","author":"Dalmau","year":"2019","journal-title":"Lancet Neurol."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/4\/462\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T22:46:03Z","timestamp":1760136363000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/4\/462"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,29]]},"references-count":46,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2022,4]]}},"alternative-id":["brainsci12040462"],"URL":"https:\/\/doi.org\/10.3390\/brainsci12040462","relation":{},"ISSN":["2076-3425"],"issn-type":[{"value":"2076-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,29]]}}}