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Extra-autonomic manifestations in autoimmune autonomic ganglionopathy: a Japanese survey
  1. Shunya Nakane1,2,
  2. Akihiro Mukaino1,
  3. Yasuhiro Maeda3,4,5,
  4. Osamu Higuchi3,
  5. Hidenori Matsuo4,
  6. Yukio Ando1
  1. 1Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
  2. 2Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital, Kumamoto, Japan
  3. 3Department of Clinical Research, National Hospital Organization Nagasaki Kawatana Medical Center, Nagasaki, Japan
  4. 4Department of Neurology, National Hospital Organization Nagasaki Kawatana Medical Center, Nagasaki, Japan
  5. 5Department of Neuroimmunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
  1. Correspondence to Dr Shunya Nakane, Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital, 1-1-1, Honjo, Chuo-ku, Kumamoto 860-8556, Japan; nakaneshunya{at}

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Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to autonomic failure. The disorder is associated with autoantibodies (Abs) to the ganglionic nicotinic acetylcholine receptor (gAChR).1 Previously, we developed a luciferase immunoprecipitation systems (LIPS) protocol to aid in the diagnosis of AAG based on serum levels of immunoglobulin G (IgG) to the α3 and β4 gAChR subunits.2 We subsequently observed that AAG is associated with an overrepresentation of autoimmune diseases and endocrine disorders.2 Furthermore, Hayashi et al3 reviewed 29 Japanese case reports of AAG and pandysautonomia and observed that Japanese patients with AAG tended to exhibit additional coughing episodes/psychiatric symptoms when compared to patients in Western countries. Gibbons et al4 reported some patients with AAG demonstrated reversible cognitive impairment. The present study aims to elucidate the prevalence of extra-autonomic manifestations including psychiatric symptoms in 80 Japanese patients with AAG who were seropositive for anti-gAChR Abs.

Materials and methods

Ethical approval

All participants provided written, informed consent to participate in the study. The Ethics Committee of Nagasaki Kawatana Medical Center approved this study.

Study population

Serum samples from 946 patients with autonomic symptoms were obtained from general and teaching hospitals throughout Japan between January 2012 and April 2016 (mean age: 51±22 years; 488 men and 458 women). Clinical diagnoses were …

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  • Contributors SN, AM and YA conceived and designed the experiments. AM, YM and OH performed the experiments. SN, AM, OH, HM and YA collected the samples and summarised the cases. SN, AM and YA analysed the data. SN, AM and YA drafted the manuscript.

  • Funding This study was supported by the Ministry of Health, Labor, and Welfare, Japan, and the Ministry of Education, Culture, Sports, Science, and Technology of Japan (JSPS KAKENHI grant number 25461305).

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.