Department of Neurology, Faculty of Medicine, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan.
Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University, Saga 849-8501, Japan.
Int J Mol Sci. 2024 Feb 15;25(4):2296. doi: 10.3390/ijms25042296.
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.
自身免疫性自主神经节病（AAG）是一种自主神经衰竭疾病，由节细胞乙酰胆碱受体（gAChR）自身抗体引起。虽然自身抗体的检测对于将该疾病与其他表现为自主功能障碍的神经病变区分开来很重要，但其他因素对于准确诊断也很重要。在这里，我们全面回顾了 AAG 的临床特征，强调了与其他表现为自主症状的神经病变在临床病程、临床表现和实验室发现方面的差异。诊断 AAG 的第一步是仔细询问病史，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，包括自主和非自主症状的出现。当患者出现自主功能障碍时，AAG 是一种应与其他神经病变区分开来的神经病变。免疫介导的神经病变，如急性自主感觉神经病，有时难以区分，因此，应充分了解临床和实验室发现的差异。其他非神经病变情况，如体位性心动过速综合征、慢性疲劳综合征和长新冠，也表现出类似于 AAG 的症状。尽管通常具有挑战性，但应努力区分这些疾病候选者。
