Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment

ABSTRACT

Introduction: Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to autonomic failure. The disorder is associated with autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR). We subsequently reported that AAG is associated with an overrepresentation of psychiatric symptoms, sensory disturbance, autoimmune diseases, and endocrine disorders.

Area covered: The aim of this review was to describe AAG and highlight its pivotal pathophysiological aspects, clinical features, laboratory examinations, and therapeutic options.

Expert commentary: AAG is a complex neuroimmunological disease, these days considered as an autonomic failure with extra-autonomic manifestations (and various limited forms). Further comprehension of the pathophysiology of this disease is required, especially the mechanisms of the extra-autonomic manifestations should be elucidated. There is the possibility that the co-presence of antibodies that were directed against the other subunits in both the central and peripheral nAChRs in the serum of the AAG patients. Some patients improve with immunotherapies such as IVIg and/or corticosteroid and/or plasma exchange. ¹²³I-MIBG myocardial scintigraphy may be a useful tool to monitor the therapeutic effects of immunotherapies.

KEYWORDS:

• Ganglionic acetylcholine receptor
• autoantibodies
• autoimmune autonomic ganglionopathy
• extra-autonomic manifestations
• immunotherapy

Acknowledgments

The authors are grateful to Drs Masataka Umeda, Kunihiro Ichinose, Hideki Nakamura, Hitomi Minami, Hajime Isomoto, Akio Ido, Kiyoshi Migita, Kazuhiko Nakao, and Atsushi Kawakami for useful discussions. The authors are indebted to members of Kumamoto University Hospital Department of Neurology and Nagasaki Kawatana Medical center Department of Neurology for discussing some issues for this study and to Yuka Okumura, Keiko Hida, Haruna Akaishi, and Haruka Ikezaki for providing excellent secretarial support.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This research was supported by AMED under Grant Number 18dk0310069h0003, and JSPS KAKENHI Grant Number 25461305 and 16K09695.