Nakane Shunya
Department of Neurology, Faculty of Medicine, Academic Research Division, University of Toyama.
Brain Nerve. 2024 May;76(5):562-568. doi: 10.11477/mf.1416202644.
Autoimmune autonomic ganglionopathy (AAG) and acute autonomic sensory neuropathy (AASN) are immune-mediated neuropathies that affect the autonomic and/or dorsal root ganglia. Autoantibodies against the nicotinic ganglionic acetylcholine receptor (gAChR) detected in the sera of patients with AAG play a key role in the pathogenesis of this condition. Notably, gAChR antibodies are not detected in the sera of patients with AASN. Currently, AAG and AASN are not considered to be on the same spectrum with regard to disease concept based on clinical symptoms and laboratory findings. However, extra-autonomic brain symptoms (including psychiatric symptoms and personality changes) and endocrine disorders occur in both diseases, which suggests shared pathophysiology between the two conditions.
自身免疫性自主神经节病(AAG)和急性自主神经感觉神经病(AASN)是影响自主神经节和/或背根神经节的免疫介导性神经病。在AAG患者血清中检测到的抗烟碱型神经节乙酰胆碱受体(gAChR)自身抗体在该病的发病机制中起关键作用。值得注意的是,在AASN患者血清中未检测到gAChR抗体。目前,基于临床症状和实验室检查结果,在疾病概念方面,AAG和AASN不被认为属于同一谱系。然而,这两种疾病均会出现自主神经以外的脑部症状(包括精神症状和人格改变)以及内分泌紊乱,这表明二者存在共同的病理生理机制。