Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients

2015 Fiscal Year Final Research Report

• Project/Area Number: 25461305
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Kumamoto University (2015); Nagasaki Kawatana Medical Center (2013-2014)
• Principal Investigator: Nakane Shunya 熊本大学, 医学部附属病院, その他 (70398022)
• Co-Investigator(Kenkyū-buntansha): HIGUCHI Osamu 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 (50361720)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Keywords: 神経内科学 / 神経免疫学 / 自律神経系 / 自己抗体

Outline of Final Research Achievements

Autoimmune autonomic ganglionopathy (AAG) is a rare acquired channelopathy that is characterized by pandysautonomia, in which autoantibodies to ganglionic nicotinic acetylcholine receptors (gAChR) may play a central role. Here, we developed luciferase immunoprecipitation systems (LIPS) to diagnose AAG based on IgGs to both the α3 and β4 gAChR subunits in patient serum. We reviewed the serological and clinical data of 50 Japanese patients who were diagnosed with AAG. With the LIPS testing, we detected anti-α3 and -β4 gAChR antibodies in 48% (24/50) of the patients. A gradual mode of onset was more common in the seropositive group than in the seronegative group. Patients with AAG frequently have orthostatic hypotension and upper and lower gastrointestinal tract symptoms, with or without anti-gAChR. In addition, we found a significant overrepresentation of　endocrinological abnormalities as an occasional complication of AAG.

Free Research Field

神経内科学