A Japanese case of autoimmune autonomic ganglionopathy (AAG) and a review of AAG cases in Japan

Abstract

A 29-year-old woman presented with acute, pure autonomic (both sympathetic and parasympathetic) failure and positive antibody to ganglionic nicotinic acetylcholine receptor; the diagnosis was autoimmune autonomic ganglionopathy (AAG). She had typical symptoms of AAG, although the patient also had coughing episodes and psychiatric symptoms, which are not typical of AAG in Western countries but are common in AAG cases in Japan. In a review of the Japanese literature, 29 cases of AAG had been reported. AAG patients in Japan were younger and more male predominant than in Western countries. Of the patients in these 29 cases, 10 (34.5%) had coughing episodes and 12 (41.4%) had psychiatric symptoms.