{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T01:38:20Z","timestamp":1789609100805,"version":"build-2803163510"},"reference-count":125,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2024,2,15]],"date-time":"2024-02-15T00:00:00Z","timestamp":1707955200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Health Labour Sciences Research Grant from the Ministry of Health, Labour and Welfare, Japan","doi-asserted-by":"publisher","award":["22FC1007"],"award-info":[{"award-number":["22FC1007"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Autoimmune autonomic ganglionopathy (AAG) is a disease of autonomic failure caused by ganglionic acetylcholine receptor (gAChR) autoantibodies. Although the detection of autoantibodies is important for distinguishing the disease from other neuropathies that present with autonomic dysfunction, other factors are important for accurate diagnosis. Here, we provide a comprehensive review of the clinical features of AAG, highlighting differences in clinical course, clinical presentation, and laboratory findings from other neuropathies presenting with autonomic symptoms. The first step in diagnosing AAG is careful history taking, which should reveal whether the mode of onset is acute or chronic, followed by an examination of the time course of disease progression, including the presentation of autonomic and extra-autonomic symptoms. AAG is a neuropathy that should be differentiated from other neuropathies when the patient presents with autonomic dysfunction. Immune-mediated neuropathies, such as acute autonomic sensory neuropathy, are sometimes difficult to differentiate, and therefore, differences in clinical and laboratory findings should be well understood. Other non-neuropathic conditions, such as postural orthostatic tachycardia syndrome, chronic fatigue syndrome, and long COVID, also present with symptoms similar to those of AAG. Although often challenging, efforts should be made to differentiate among the disease candidates.<\/jats:p>","DOI":"10.3390\/ijms25042296","type":"journal-article","created":{"date-parts":[[2024,2,15]],"date-time":"2024-02-15T03:57:29Z","timestamp":1707969449000},"page":"2296","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis"],"prefix":"10.3390","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1958-095X","authenticated-orcid":false,"given":"Shunya","family":"Nakane","sequence":"first","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haruki","family":"Koike","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga 849-8501, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiro","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuji","family":"Nakatsuji","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,2,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1806","DOI":"10.1212\/WNL.50.6.1806","article-title":"Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes","volume":"50","author":"Vernino","year":"1998","journal-title":"Neurology"},{"key":"ref_2","doi-asserted-by":"crossref","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. 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