{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T00:20:02Z","timestamp":1778718002980,"version":"3.51.4"},"reference-count":29,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T00:00:00Z","timestamp":1680652800000},"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. Immunol."],"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>An analysis of the clinical features of autoimmune encephalitis accompanied by anti-amphiphysin antibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The data of encephalitis patients with anti-amphiphysin antibodies were retrospectively evaluated, including demographics, neurological and laboratory findings, imaging, treatment, and prognostic predictions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Ten patients aged between 29 and 78 years (median age 52 years) were included. The male: female ratio was 4:6. Limbic encephalitis was found in nine patients while epileptic seizures were present in seven patients. All patients showed anti-amphiphysin antibody positivity in sera while one ninth was positive for CSF antibody. The EEG findings were abnormal, including reductions in background activity, and the presence of diffuse slow waves, sharp waves, and spikes and waves. Five patients showed signs of increased T2 signals in the medial temporal lobe on MRI while PET showed either hyper- or hypo-metabolic changes in several brain regions, including the temporal lobe, hippocampus, basal ganglia, frontal and parietal cortices. Nine of ten patients were treated with immunotherapy, with improvements of varying degrees. There was a significant reduction in seizure frequency, and all patients were seizure-free at last follow-up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Autoimmune encephalitis with anti-amphiphysin antibodies has a variety of clinical manifestations. The most common symptom is limbic encephalitis. Although relief from seizures can be achieved relatively easily, many patients suffer psychiatric, cognitive, and sleep sequelae. The disease was found to be associated with a lower incidence of cancer than has been previously reported for paraneoplastic neurologic syndromes.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fimmu.2023.1084883","type":"journal-article","created":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T05:12:07Z","timestamp":1680671527000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":15,"title":["Anti-amphiphysin encephalitis: Expanding the clinical spectrum"],"prefix":"10.3389","volume":"14","author":[{"given":"Yueqian","family":"Sun","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoxiao","family":"Qin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danxia","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ziqi","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yudi","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qun","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,4,5]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1016\/s1474-4422(15)00401-9","article-title":"A clinical approach to diagnosis of autoimmune encephalitis","volume":"15","author":"Graus","year":"2016","journal-title":"Lancet Neurol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25131","article-title":"Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis","volume":"83","author":"Dubey","year":"2018","journal-title":"Ann Neurol"},{"key":"B3","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.jaut.2018.10.014","article-title":"Prognosticating autoimmune encephalitis: A systematic review","volume":"96","author":"Broadley","year":"2019","journal-title":"J Autoimmun"},{"key":"B4","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1002\/ana.21050","article-title":"Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma","volume":"61","author":"Dalmau","year":"2007","journal-title":"Ann Neurol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2018.09.009","article-title":"Pathogenic role of autoantibodies against inhibitory synapses","volume":"1701","author":"Pruss","year":"2018","journal-title":"Brain Res"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1111\/j.1398-9219.2004.00198.x","article-title":"Amphiphysin is a component of clathrin coats formed during synaptic vesicle recycling at the lamprey giant synapse","volume":"5","author":"Evergren","year":"2004","journal-title":"Traffic."},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1212\/nxi.0000000000000404","article-title":"Improving the antibody-based evaluation of autoimmune encephalitis","volume":"4","author":"McCracken","year":"2017","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.66.2.214","article-title":"Anti-amphiphysin I antibodies in patients with paraneoplastic neurological disorders associated with small cell lung carcinoma","volume":"66","author":"Saiz","year":"1999","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1097\/CND.0b013e31826f0d99","article-title":"Lower extremity predominant stiff-person syndrome and limbic encephalitis with amphiphysin antibodies in breast cancer","volume":"14","author":"Krishna","year":"2012","journal-title":"J Clin Neuromuscul Dis"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2014.07.011","article-title":"Non-stiff anti-amphiphysin syndrome: Clinical manifestations and outcome after immunotherapy","volume":"274","author":"Moon","year":"2014","journal-title":"J Neuroimmunol."},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1159\/000092307","article-title":"Paraneoplastic rhombencephalitis and brachial plexopathy in two cases of amphiphysin auto-immunity","volume":"55","author":"Coppens","year":"2006","journal-title":"Eur Neurol"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1159\/000331592","article-title":"Stiff leg syndrome and myelitis with anti-amphiphysin antibodies: A common physiopathology","volume":"66","author":"Chamard","year":"2011","journal-title":"Eur Neurol"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31821f468f","article-title":"Paraneoplastic isolated myelopathy: clinical course and neuroimaging clues","volume":"76","author":"Flanagan","year":"2011","journal-title":"Neurology."},{"key":"B14","doi-asserted-by":"publisher","first-page":"196","DOI":"10.1159\/000057898","article-title":"Autoantibodies to amphiphysin I and amphiphysin II in a patient with sensory-motor neuropathy","volume":"47","author":"Perego","year":"2002","journal-title":"Eur Neurol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.02066","article-title":"Innate immunity in the central nervous system: A missing piece of the autoimmune encephalitis puzzle","volume":"10","author":"Wesselingh","year":"2019","journal-title":"Front Immunol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(05)76151-1","article-title":"Stiff-man syndrome and its variants","volume":"353","author":"Shaw","year":"1999","journal-title":"Lancet."},{"key":"B17","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1002\/ana.20529","article-title":"Amphiphysin autoimmunity: Paraneoplastic accompaniments","volume":"58","author":"Pittock","year":"2005","journal-title":"Ann Neurol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000327342.58936.e0","article-title":"Stiff-person syndrome with amphiphysin antibodies: distinctive features of a rare disease","volume":"71","author":"Murinson","year":"2008","journal-title":"Neurology."},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000008472","article-title":"Amphiphysin-IgG autoimmune neuropathy: A recognizable clinicopathologic syndrome","volume":"93","author":"Dubey","year":"2019","journal-title":"Neurology."},{"key":"B20","doi-asserted-by":"publisher","first-page":"e24312","DOI":"10.1097\/MD.0000000000024312","article-title":"Involuntary movement in stiff-person syndrome with amphiphysin antibodies: A case report","volume":"100","author":"Xie","year":"2021","journal-title":"Med (Baltimore)."},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2016.0133","article-title":"Clinical and immunologic investigations in patients with stiff-person spectrum disorder","volume":"73","author":"Martinez-Hernandez","year":"2016","journal-title":"JAMA Neurol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1007\/s13760-015-0534-9","article-title":"Longitudinally extensive transverse myelitis associated with amphiphysin autoimmunity and breast cancer: a paraneoplastic accompaniment","volume":"116","author":"Galassi","year":"2016","journal-title":"Acta Neurol Belg."},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000001014","article-title":"Updated diagnostic criteria for paraneoplastic neurologic syndromes","volume":"8","author":"Graus","year":"2021","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp136","article-title":"The pattern and diagnostic criteria of sensory neuronopathy: a case-control study","volume":"132","author":"Camdessanche","year":"2009","journal-title":"Brain."},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1111\/j.1529-8027.2012.00411.x","article-title":"A clinical pattern-based etiological diagnostic strategy for sensory neuronopathies: a French collaborative study","volume":"17","author":"Camdessanche","year":"2012","journal-title":"J Peripher Nerv Syst"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1016\/s0140-6736(05)66376-3","article-title":"Paraneoplastic stiff-person syndrome: Passive transfer to rats by means of IgG antibodies to amphiphysin","volume":"365","author":"Sommer","year":"2005","journal-title":"Lancet"},{"key":"B27","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/j.jaut.2019.04.007","article-title":"The neurological syndromes associated with glutamic acid decarboxylase antibodies","volume":"101","author":"Baizabal-Carvallo","year":"2019","journal-title":"J Autoimmun"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1212\/wnl.51.1.250","article-title":"Atypical stiff-person syndrome with spinal MRI findings, amphiphysin autoantibodies, and immunosuppression","volume":"51","author":"Schmierer","year":"1998","journal-title":"Neurology."},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebr.2022.100534","article-title":"All's well that ends well? long-term course of a patient with anti-amphiphysin associated limbic encephalitis","volume":"18","author":"Taube","year":"2022","journal-title":"Epilepsy Behav Rep"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2023.1084883\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T05:12:09Z","timestamp":1680671529000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2023.1084883\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,5]]},"references-count":29,"alternative-id":["10.3389\/fimmu.2023.1084883"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2023.1084883","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,5]]},"article-number":"1084883"}}