PMID- 28214088 OWN - NLM STAT- MEDLINE DCOM- 20170523 LR - 20181202 IS - 1532-2653 (Electronic) IS - 0967-5868 (Linking) VI - 39 DP - 2017 May TI - A case of seropositive autoimmune autonomic ganglionopathy with diffuse esophageal spasm. PG - 90-92 LID - S0967-5868(16)31266-8 [pii] LID - 10.1016/j.jocn.2017.01.027 [doi] AB - 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-alpha3-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. CI - Copyright (c) 2017 Elsevier Ltd. All rights reserved. FAU - Morimoto, Nobutoshi AU - Morimoto N AD - Department of Neurology, Kagawa Prefectural Central Hospital, Japan. Electronic address: morinobu@cc.okayama-u.ac.jp. FAU - Takahashi, Sakuma AU - Takahashi S AD - Department of Gastroenterology, Kagawa Prefectural Central Hospital, Japan. FAU - Inaba, Tomoki AU - Inaba T AD - Department of Gastroenterology, Kagawa Prefectural Central Hospital, Japan. FAU - Takamiya, Motonori AU - Takamiya M AD - Department of Neurology, Kagawa Prefectural Central Hospital, Japan. FAU - Kageyama, Yasuhiko AU - Kageyama Y AD - Department of Neurology, Kagawa Prefectural Central Hospital, Japan. FAU - Morimoto, Mizuki AU - Morimoto M AD - Department of Neurology, Kagawa Prefectural Central Hospital, Japan. FAU - Takahashi, Yoshiaki AU - Takahashi Y AD - Department of Neurology, Kagawa Prefectural Central Hospital, Japan; Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Japan. FAU - Nishimura, Hirotake AU - Nishimura H AD - Department of Pathology, Kawasaki Medical School, Japan. FAU - Nakane, Shunya AU - Nakane S AD - Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan. FAU - Abe, Koji AU - Abe K AD - Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Japan. LA - eng PT - Case Reports PT - Journal Article DEP - 20170214 PL - Scotland TA - J Clin Neurosci JT - Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia JID - 9433352 RN - 0 (Autoantibodies) SB - IM MH - Aged MH - Autoantibodies/blood MH - Autonomic Nervous System Diseases/blood/*complications/*diagnostic imaging MH - Esophageal Spasm, Diffuse/blood/*complications/*diagnostic imaging MH - Female MH - Ganglia, Autonomic/*diagnostic imaging MH - Humans OTO - NOTNLM OT - Anti-ganglionic acetylcholine receptor alpha3 antibody OT - Autoimmune autonomic ganglionopathy (AAG) OT - Diffuse esophageal spasm (DES) OT - Intravenous immunoglobulin therapy (IVIg) EDAT- 2017/02/19 06:00 MHDA- 2017/05/24 06:00 CRDT- 2017/02/19 06:00 PHST- 2016/11/16 00:00 [received] PHST- 2017/01/22 00:00 [accepted] PHST- 2017/02/19 06:00 [pubmed] PHST- 2017/05/24 06:00 [medline] PHST- 2017/02/19 06:00 [entrez] AID - S0967-5868(16)31266-8 [pii] AID - 10.1016/j.jocn.2017.01.027 [doi] PST - ppublish SO - J Clin Neurosci. 2017 May;39:90-92. doi: 10.1016/j.jocn.2017.01.027. Epub 2017 Feb 14.