title: A case of seropositive autoimmune autonomic ganglionopathy with diffuse esophageal spasm.
abstract: Autoimmune autonomic ganglionopathy (AAG) is an immune-mediated disorder that leads to various autonomic failures associated with anti-ganglionic acetylcholine receptor antibodies (anti-gAChR-Abs). Diffuse esophageal spasm (DES) is an uncommon esophageal motility disorder. We herein report the case of a 68-year-old woman with DES as a partial symptom of AAG. She presented with chronic esophageal transit failure, constipation, and numbness of the hands and feet, Adie's pupil, thermal hypoalgesia, and decreased deep tendon reflexes. Right sural nerve biopsy showed significantly decreased numbers of small myelinated fibers. Barium swallowing X-ray showed repetitive simultaneous contractions indicating DES in the esophagus. Gastrointestinal endoscopy and CT image showed a dilated esophageal lumen and liquid effusion. Simultaneously, serum anti-gAChR-α3-Ab indicating AAG was detected. After pulse intravenous methylprednisolone (IVMP) and intravenous immunoglobulin therapy (IVIg), the bolus progression and liquid effusion improved, suggesting that DES is an important gastrointestinal symptom of AAG.
authors.affiliation: Department of Neurology, Kagawa Prefectural Central Hospital, Japan. Electronic address: morinobu@cc.okayama-u.ac.jp.
authors.forename: Nobutoshi
authors.initials: N
authors.lastname: Morimoto
authors.affiliation: Department of Gastroenterology, Kagawa Prefectural Central Hospital, Japan.
authors.forename: Sakuma
authors.initials: S
authors.lastname: Takahashi
authors.affiliation: Department of Gastroenterology, Kagawa Prefectural Central Hospital, Japan.
authors.forename: Tomoki
authors.initials: T
authors.lastname: Inaba
authors.affiliation: Department of Neurology, Kagawa Prefectural Central Hospital, Japan.
authors.forename: Motonori
authors.initials: M
authors.lastname: Takamiya
authors.affiliation: Department of Neurology, Kagawa Prefectural Central Hospital, Japan.
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Kageyama
authors.affiliation: Department of Neurology, Kagawa Prefectural Central Hospital, Japan.
authors.forename: Mizuki
authors.initials: M
authors.lastname: Morimoto
authors.affiliation: Department of Neurology, Kagawa Prefectural Central Hospital, Japan; Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Takahashi
authors.affiliation: Department of Pathology, Kawasaki Medical School, Japan.
authors.forename: Hirotake
authors.initials: H
authors.lastname: Nishimura
authors.affiliation: Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan.
authors.forename: Shunya
authors.initials: S
authors.lastname: Nakane
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001342:Autonomic Nervous System Diseases / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging*; D015155:Esophageal Spasm, Diffuse / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging*; D005260:Female; D005725:Ganglia, Autonomic / Q000000981:diagnostic imaging*; D006801:Humans
chemical_list: D001323:Autoantibodies
keywords: Anti-ganglionic acetylcholine receptor α3 antibody; Autoimmune autonomic ganglionopathy (AAG); Diffuse esophageal spasm (DES); Intravenous immunoglobulin therapy (IVIg)