Case of autoimmune autonomic ganglionopathy manifesting anhidrosis. Issue 10 (16th June 2017)

• Record Type: Journal Article
• Authors: Yoshifuku, Asuka; Yoneda, Koichi; Sakiyama, Yusuke; Higuchi, Osamu; Nakane, Shunya; Kanekura, Takuro
• Is Part Of: Journal of dermatology. Volume 44:Issue 10(2017)
• Page Start: 1160
• Page End: 1163
• Publication Date: 2017-06-16
• Subjects: anhidrosis -- autoimmune autonomic ganglionopathy -- autoimmune disease -- ganglionic acetylcholine receptor -- Horner's syndrome; Dermatology -- Periodicals; Dermatology -- Japan -- Periodicals; Skin -- Diseases -- Periodicals; 616.5005
• DOI: 10.1111/1346-8138.13870
• Languages: English
• ISSNs: 0385-2407

Abstract

Abstract: Autoimmune autonomic ganglionopathy (AAG), clinically characterized by gastrointestinal dysmotility, orthostatic hypotension and tonic pupils, is an idiopathic acquired disorder of the autonomic nervous system elicited by antibodies against ganglionic acetylcholine receptor (gAChR). We encountered a 60‐year‐old man who presented with severe anhidrosis, difficulty in thermoregulation, orthostatic hypotension, gastrointestinal dysmotility, tonic pupils and ptosis. Histologically, an anhidrotic skin sample was normal. Routine laboratory examinations of blood, urine and cerebrospinal fluid returned no abnormal findings. Serological examination revealed antibodies against α3 and β4 subunits of gAChR. The diagnosis was AAG. As sudomotor dysfunction reflects ganglionic neuropathy in AAG, we concluded that his anhidrosis was attributable to AAG. Anhidrosis is an important clue for the diagnosis of AAG, a rare neurological disorder.