Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
J Dermatol. 2017 Oct;44(10):1160-1163. doi: 10.1111/1346-8138.13870. Epub 2017 Jun 16.
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.
自身免疫性自主神经节病 (AAG)，临床上以胃肠动力障碍、体位性低血压和紧张性瞳孔为特征，是一种由抗神经节乙酰胆碱受体 (gAChR) 抗体引起的特发性获得性自主神经系统疾病。我们遇到一位 60 岁男性，表现为严重无汗、体温调节困难、体位性低血压、胃肠动力障碍、紧张性瞳孔和上睑下垂。组织学上，无汗皮肤样本正常。血液、尿液和脑脊液的常规实验室检查均未见异常发现。血清学检查显示针对 gAChR 的α3 和β4 亚单位的抗体。诊断为 AAG。由于出汗功能障碍反映了 AAG 中的神经节性神经病，我们得出结论，他的无汗是由 AAG 引起的。无汗是 AAG 的一个重要诊断线索，AAG 是一种罕见的神经疾病。
