PDF(Pubmed)
Abstract：
Autoimmune autonomic ganglionopathy (AAG) is a disease of autonomic failure caused by ganglionic acetylcholine receptor (gAChR) autoantibodies. Although the detection of autoantibodies is important for distinguishing the disease from other neuropathies that present with autonomic dysfunction, other factors are important for accurate diagnosis. Here, we provide a comprehensive review of the clinical features of AAG, highlighting differences in clinical course, clinical presentation, and laboratory findings from other neuropathies presenting with autonomic symptoms. The first step in diagnosing AAG is careful history taking, which should reveal whether the mode of onset is acute or chronic, followed by an examination of the time course of disease progression, including the presentation of autonomic and extra-autonomic symptoms. AAG is a neuropathy that should be differentiated from other neuropathies when the patient presents with autonomic dysfunction. Immune-mediated neuropathies, such as acute autonomic sensory neuropathy, are sometimes difficult to differentiate, and therefore, differences in clinical and laboratory findings should be well understood. Other non-neuropathic conditions, such as postural orthostatic tachycardia syndrome, chronic fatigue syndrome, and long COVID, also present with symptoms similar to those of AAG. Although often challenging, efforts should be made to differentiate among the disease candidates.
摘要：
自身免疫性自主神经节病变（AAG）是由神经节乙酰胆碱受体（gAChR）自身抗体引起的自主神经功能衰竭的疾病。尽管自身抗体的检测对于区分疾病与其他伴有自主神经功能障碍的神经病很重要，其他因素对准确诊断很重要。这里,我们对AAG的临床特征进行了全面的综述，突出临床过程中的差异，临床表现,以及其他表现出自主神经症状的神经病的实验室发现。诊断AAG的第一步是仔细记录历史，这应该揭示发病模式是急性还是慢性，然后检查疾病进展的时间过程，包括自主神经和自主神经外症状的表现。AAG是一种神经病，当患者出现自主神经功能障碍时，应与其他神经病区分开。免疫介导的神经病,如急性自主神经感觉神经病变，有时很难区分，因此,临床和实验室检查结果的差异应该得到很好的理解.其他非神经性疾病，比如体位性心动过速综合征，慢性疲劳综合征,和长长的COVID，也存在与AAG相似的症状。虽然经常具有挑战性，应努力区分候选疾病。
