Autoimmune autonomic ganglionopathy: an update on diagnosis and treatment

被引:27
作者
Nakane, Shunya [1 ,2 ]
Mukaino, Akihiro [1 ,2 ]
Higuchi, Osamu [3 ]
Watari, Mari [1 ]
Maeda, Yasuhiro [3 ]
Yamakawa, Makoto [1 ]
Nakahara, Keiichi [1 ]
Takamatsu, Koutaro [1 ]
Matsuo, Hidenori [3 ]
Ando, Yukio [1 ]
机构
[1] Kumamoto Univ, Grad Sch Med Sci, Dept Neurol, Kumamoto, Japan
[2] Kumamoto Univ Hosp, Dept Mol Neurol & Therapeut, Kumamoto, Japan
[3] Nagasaki Kawatana Med Ctr, Dept Neurol & Clin Res, Nagasaki, Japan
关键词
Ganglionic acetylcholine receptor; autoantibodies; autoimmune autonomic ganglionopathy; extra-autonomic manifestations; immunotherapy; NICOTINIC ACETYLCHOLINE-RECEPTORS; NEUROGENIC ORTHOSTATIC HYPOTENSION; POSTURAL TACHYCARDIA SYNDROME; AUTOIMMUNOREACTIVE IGGS; ACUTE PANDYSAUTONOMIA; GAUSSIA LUCIFERASE; MYASTHENIA-GRAVIS; AUTOANTIBODIES; NEUROPATHY; SYSTEM;
D O I
10.1080/14737175.2018.1540304
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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. I-123-MIBG myocardial scintigraphy may be a useful tool to monitor the therapeutic effects of immunotherapies.
引用
收藏
页码:953 / 965
页数:13
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