{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T16:05:49Z","timestamp":1753891549048,"version":"3.41.2"},"reference-count":20,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T00:00:00Z","timestamp":1736467200000},"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 autonomic ganglionopathy (AAG) is a rare and acquired immune-mediated disease that leads to wide autonomic failure, mainly characterized by orthostatic hypotension, gastrointestinal dysfunction, anhidrosis and poorly reactive pupils. This disorder is usually associated with autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR-Ab). In this study, we describe a case of a gAChR-Ab-positive AAG patient with two therapeutic stages. The patient responded well after the first stage of methylprednisolone pulse therapy and subsequent low-dose prednisone. However, AAG relapsed after stopping oral prednisone. In the second stage, repeated methylprednisolone pulse therapy was less effective than before. Fortunately, multiple plasma exchange treatments improved the patient\u2019s symptoms. In the end, low-dose oral prednisone and mycophenolate mofetil provided significant improvement in this patient during long-term follow-up. AAG is a relatively rare neuroimmunological disease with insidious onset and confused clinical features, while it responds well to the conventional immunotherapy, and some patients may require a long-term immunotherapy. Emphasizing the importance of early detection and treatment in clinical practice. Moreover, it should be noted that the reduction and withdrawal of immunosuppressants should be slow and cautious.<\/jats:p>","DOI":"10.3389\/fneur.2024.1533840","type":"journal-article","created":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T05:10:32Z","timestamp":1736485832000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["A case of relapsed gAChR-positive autoimmune autonomic ganglionopathy treated by plasma exchange and mycophenolate mofetil"],"prefix":"10.3389","volume":"15","author":[{"given":"Zhijie","family":"Lu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaojie","family":"Cao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mingyu","family":"Wang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fang","family":"Peng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lin","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zegang","family":"Yin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Baiyang","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jin","family":"Fan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mingjie","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,1,10]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"102988","DOI":"10.1016\/j.autrev.2021.102988","article-title":"Autoimmune autonomic Ganglionopathy: ganglionic acetylcholine receptor autoantibodies","volume":"21","author":"Urriola","year":"2022","journal-title":"Autoimmun Rev"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1016\/j.autneu.2008.09.005","article-title":"Autonomic ganglia, acetylcholine receptor antibodies, and autoimmune Ganglionopathy","volume":"146","author":"Vernino","year":"2009","journal-title":"Auton Neurosci"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"953","DOI":"10.1080\/14737175.2018.1540304","article-title":"Autoimmune autonomic Ganglionopathy: an update on diagnosis and treatment","volume":"18","author":"Nakane","year":"2018","journal-title":"Expert Rev Neurother"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"752","DOI":"10.1002\/ana.10556","article-title":"The Spectrum of autoimmune autonomic neuropathies","volume":"53","author":"Klein","year":"2003","journal-title":"Ann Neurol"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1007\/s10286-019-00611-1","article-title":"Autoimmune autonomic neuropathies and Ganglionopathies: epidemiology, pathophysiology, and therapeutic advances","volume":"29","author":"Golden","year":"2019","journal-title":"Clin Auton Res"},{"key":"ref6","first-page":"355","article-title":"Pure Pan-Dysautonomia with recovery","volume":"94","author":"Young","year":"1969","journal-title":"Trans Am Neurol Assoc"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"725","DOI":"10.1136\/jnnp.46.8.725","article-title":"Acute pandysautonomia and severe sensory deficit with poor recovery. A clinical, neurophysiological and pathological case study","volume":"46","author":"Fagius","year":"1983","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1056\/NEJM200009213431204","article-title":"Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies","volume":"343","author":"Vernino","year":"2000","journal-title":"N Engl J Med"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"9742","DOI":"10.1172\/JCI179742","article-title":"Myasthenia gravis: the future is here","volume":"134","author":"Kaminski","year":"2024","journal-title":"J Clin Invest"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1016\/j.autneu.2008.11.013","article-title":"Antibody titers predict clinical features of autoimmune autonomic Ganglionopathy","volume":"146","author":"Gibbons","year":"2009","journal-title":"Auton Neurosci"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1016\/j.jneuroim.2008.03.017","article-title":"Characterization of ganglionic acetylcholine receptor autoantibodies","volume":"197","author":"Vernino","year":"2008","journal-title":"J Neuroimmunol"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"e0118312","DOI":"10.1371\/journal.pone.0118312","article-title":"Clinical features of autoimmune autonomic Ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients","volume":"10","author":"Nakane","year":"2015","journal-title":"PLoS One"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"102403","DOI":"10.1016\/j.jaut.2020.102403","article-title":"A comprehensive analysis of the clinical characteristics and laboratory features in 179 patients with autoimmune autonomic Ganglionopathy","volume":"108","author":"Nakane","year":"2020","journal-title":"J Autoimmun"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1001\/archneurol.2011.2143","article-title":"A unique manifestation of pupillary fatigue in autoimmune autonomic Ganglionopathy","volume":"69","author":"Muppidi","year":"2012","journal-title":"Arch Neurol"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1002\/mus.27048","article-title":"Autonomic neuropathies","volume":"63","author":"Kaur","year":"2021","journal-title":"Muscle Nerve"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"262","DOI":"10.1186\/s40425-019-0748-0","article-title":"Seronegative autoimmune autonomic Ganglionopathy from dual immune checkpoint inhibition in a patient with metastatic melanoma","volume":"7","author":"Gao","year":"2019","journal-title":"J Immunother Cancer"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"2296","DOI":"10.3390\/ijms25042296","article-title":"Autoimmune autonomic neuropathy: from pathogenesis to diagnosis","volume":"25","author":"Nakane","year":"2024","journal-title":"Int J Mol Sci"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1007\/s10286-009-0012-7","article-title":"Autoimmune autonomic Ganglionopathy: treatment by plasma exchanges and rituximab","volume":"19","author":"Imrich","year":"2009","journal-title":"Clin Auton Res"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"2002","DOI":"10.1212\/WNL.0b013e3181a92b52","article-title":"Efficacy of immunotherapy in seropositive and seronegative putative autoimmune autonomic Ganglionopathy","volume":"72","author":"Iodice","year":"2009","journal-title":"Neurology"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1177\/17562864221110048","article-title":"Effectiveness of treatment for 31 patients with seropositive autoimmune autonomic Ganglionopathy in Japan","volume":"15","author":"Hayashi","year":"2022","journal-title":"Ther Adv Neurol Disord"}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2024.1533840\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T05:10:35Z","timestamp":1736485835000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2024.1533840\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,10]]},"references-count":20,"alternative-id":["10.3389\/fneur.2024.1533840"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2024.1533840","relation":{},"ISSN":["1664-2295"],"issn-type":[{"type":"electronic","value":"1664-2295"}],"subject":[],"published":{"date-parts":[[2025,1,10]]},"article-number":"1533840"}}