title: Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients.
abstract: 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. Radioimmunoprecipitation (RIP) assays have been used for the sensitive detection of autoantibodies to gAChR in the serum of patients with AAG. 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. The occurrence of autonomic symptoms was not significantly different between the seropositive and seronegative group, with the exception of achalasia in three patients from the seropositive group. In addition, we found a significant overrepresentation of autoimmune diseases in the seropositive group and endocrinological abnormalities as an occasional complication of AAG. Our results demonstrated that the LIPS assay was a useful novel tool for detecting autoantibodies against gAChR in patients with AAG.
authors: Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan; Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors: Shunya
authors: S
authors: Nakane
authors: Department of Clinical Research, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors: Osamu
authors: O
authors: Higuchi
authors: Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.
authors: Michiaki
authors: M
authors: Koga
authors: Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan.
authors: Takashi
authors: T
authors: Kanda
authors: Department of Neurology, Wakayama Medical University, Wakayama, Japan.
authors: Kenya
authors: K
authors: Murata
authors: Department of Neurology, Joetsu General Hospital, Niigata, Japan.
authors: Takashi
authors: T
authors: Suzuki
authors: Department of Neurology, Saiseikai Kanagawa Prefecture Hospital, Kanagawa, Japan.
authors: Hiroko
authors: H
authors: Kurono
authors: Department of Neurology, Saiseikai Kanagawa Prefecture Hospital, Kanagawa, Japan.
authors: Masanari
authors: M
authors: Kunimoto
authors: Division of Neurology, Department of Internal Medicine 3, National Defense Medical College, Saitama, Japan.
authors: Ken-ichi
authors: K
authors: Kaida
authors: Department of Clinical Neuroscience and Neurology, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan.
authors: Akihiro
authors: A
authors: Mukaino
authors: Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors: Waka
authors: W
authors: Sakai
authors: Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors: Yasuhiro
authors: Y
authors: Maeda
authors: Department of Neurology, Nagasaki Kawatana Medical Center, Nagasaki, Japan.
authors: Hidenori
authors: H
authors: Matsuo
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood*; D020274:Autoimmune Diseases of the Nervous System / Q000175:diagnosis*; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis* / Q000276:immunology; D002648:Child; D005260:Female; D005725:Ganglia, Autonomic / Q000276:immunology; D006801:Humans; D007024:Hypotension, Orthostatic / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D015531:Radioimmunoprecipitation Assay; D011978:Receptors, Nicotinic / Q000276:immunology*; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D011978:Receptors, Nicotinic