eterogeneity and continuum of clinical features in autoimmune autonomic ganglionopathy
| Project/Area Number |
16K09695
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Kumamoto University |
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Principal Investigator |
Nakane Shunya 熊本大学, 医学部附属病院, 特任教授 (70398022)
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| Co-Investigator(Kenkyū-buntansha) |
樋口 理 独立行政法人国立病院機構長崎川棚医療センター(臨床研究部), その他の研究科, 研究員(移行) (50361720)
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| Project Period (FY) |
2016-04-01 – 2019-03-31
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| Project Status |
Completed (Fiscal Year 2018)
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| Budget Amount *help |
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2018: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2017: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2016: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
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| Keywords | 自己免疫性自律神経節障害 / 抗自律神経節アセチルコリン受容体抗体 / 多様性 / 体位性起立性頻脈症候群 / 自己免疫性消化管運動障害 / 無汗症 / 体位性頻脈症候群 / 脳神経疾患 / 内科 |
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| Outline of Final Research Achievements |
The clinical associations of anglionic acetylcholine receptor (gAChR) have not yet been described fully. It is not known whether central nervous system (CNS) involvement and endocrine disorders are the extra-autonomic features of autoimmune autonomic ganglionopathy (AAG), or whether it is related to circulating anti-gAChR antibodies (Abs). We identified prospectively 179 Abs-positive AAG patients in Japan. Luciferase immunoprecipitation systems were conducted to detect anti-gAChR Abs. Patients with AAG demonstrated widespread autonomic dysfunction. In particular, orthostatic hypotension and lower gastrointestinal tract dysfunction were frequently observed. Seropositive AAG patients showed a range of clinical features including CNS involvement (33%), endocrine disorders (15%), other autoimmune disease (30%), and tumors (10%). And most showed marked improvement in the clinical status and the levels of anti-gAChR Abs with immunotherapies.
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| Academic Significance and Societal Importance of the Research Achievements |
自己免疫性自律神経節障害は自律神経系が免疫異常の標的となる比較的新しい疾患概念である．本症では抗自律神経節アセチルコリン受容体抗体は病原性自己抗体として病態の鍵となる役割を果たす．我々は2012年にこの自己抗体測定を本邦で初めて可能とし，日本における自己免疫性自律神経節障害症例の調査に努めてきた．本症は自律神経系外の症候（中枢神経系障害，感覚障害，内分泌障害）や併存症（膠原病など），小児症例が存在することが判ってきた．この「多様性」が本症の診断しにくさ，難治化につながっていることがわかった．また慢性疲労症候群など本症と類似の症候を呈する疾患群との異同の解明も今後の研究上の重要な課題である．
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Report
(4 results)
Research Products
(26 results)
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[Journal Article] Anti-ganglionic AChR antibodies in Japanese patients with motility disorders2018
Author(s)
Mukaino A, Minami H, Isomoto H, Hamamoto H, Ihara E, Maeda Y, Higuchi O, Okanishi T, Kokudo Y, Deguchi K, Sasaki F, Ueki T, Murata KY, Yoshida T, Kinjo M, Ogawa Y, Ido A, Matsuo H, Nakao K, Nakane S
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Journal Title
J Gastroenterol
Volume: 53
Issue: 12
Pages: 1227-1240
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Immune checkpoint inhibitors in the onset of myasthenia gravis with hyperCKemia.2018
Author(s)
Koutaro Takamatsu, Shunya Nakane, Shigeaki Suzuki, Takayuki Kosaka, Satoshi Fukushima, Toshihiro Kimura, Azusa Miyashita, Akihiro Mukaino, Shiori Yamakawa, Keisuke Watanabe, Masatoshi Jinnin, Yoshihiro Komohara, Hironobu Ihn, and Yukio Ando
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Journal Title
Ann Clin Transl Neurol.
Volume: 5
Issue: 11
Pages: 1421-1427
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Autoimmune postural orthostatic tachycardia syndrome.2018
Author(s)
Watari M, Nakane S, Mukaino A, Nakajima M, Mori Y, Maeda Y, Masuda T, Takamatsu K, Kouzaki Y, Higuchi O, Matsuo H, Ando Y.
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Journal Title
Ann Clin Transl Neurol.
Volume: 5
Issue: 4
Pages: 486-492
DOI
Related Report
Peer Reviewed / Open Access
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[Presentation] Ganglionic acetylcholine receptor antibodies in autoimmune autonomic ganglionopathy: Characteristics, clinical features, and outcomes.2017
Author(s)
Nakane S, Mukaino I, Higuchi O, Maeda Y, Takamatsu K, Watari M, Kosaka T, Matsuo H, Ando Y.
Organizer
XXIII World Congress of Neurology
Related Report
Int'l Joint Research
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