Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis
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...
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| Format: | Article |
| Language: | English |
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MDPI AG
2024-02-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/25/4/2296 |
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| author | Shunya Nakane Haruki Koike Tomohiro Hayashi Yuji Nakatsuji |
| author_facet | Shunya Nakane Haruki Koike Tomohiro Hayashi Yuji Nakatsuji |
| author_sort | Shunya Nakane |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-07T22:28:14Z |
| format | Article |
| id | doaj.art-019d8b6fe53c4849948c81a920014802 |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-07T22:28:14Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
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| series | International Journal of Molecular Sciences |
| spelling | doaj.art-019d8b6fe53c4849948c81a9200148022024-02-23T15:20:29ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672024-02-01254229610.3390/ijms25042296Autoimmune Autonomic Neuropathy: From Pathogenesis to DiagnosisShunya Nakane0Haruki Koike1Tomohiro Hayashi2Yuji Nakatsuji3Department of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, JapanDivision of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga 849-8501, JapanDepartment of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, JapanDepartment of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, JapanAutoimmune 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.https://www.mdpi.com/1422-0067/25/4/2296ganglionic acetylcholine receptorautoantibodiesautoimmune autonomic ganglionopathyextra-autonomic manifestationsimmunotherapy |
| spellingShingle | Shunya Nakane Haruki Koike Tomohiro Hayashi Yuji Nakatsuji Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis International Journal of Molecular Sciences ganglionic acetylcholine receptor autoantibodies autoimmune autonomic ganglionopathy extra-autonomic manifestations immunotherapy |
| title | Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis |
| title_full | Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis |
| title_fullStr | Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis |
| title_full_unstemmed | Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis |
| title_short | Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis |
| title_sort | autoimmune autonomic neuropathy from pathogenesis to diagnosis |
| topic | ganglionic acetylcholine receptor autoantibodies autoimmune autonomic ganglionopathy extra-autonomic manifestations immunotherapy |
| url | https://www.mdpi.com/1422-0067/25/4/2296 |
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