Abstract：
We report the clinical course of three cases of anti-ganglionic acetylcholine receptor (gAChR) antibody positive auto-immune autonomic ganglionopathy (AAG) that have been followed for over 5 years. In all three cases, the symptoms improved by acute treatment, but ultimately relapsed. The first case was a female in her 20s who had a chronic history of photophobia, constipation and amenorrhea. The symptoms almost disappeared by plasma exchange, and menstruation resumed. During the course, it relapsed once after a cold. There was no recurrence of AAG during the two pregnancies. The second case was a male in his 60s who visited a hospital for the acute onset of orthostatic hypotension (OH) and psychological symptoms (infantilization and psychogenic pseudosyncope). Although IVIg was effective, it recurred frequently and was difficult to treat. However, all the symptoms disappeared eight years after the onset without any particular reasons. The third case was a female in her 80s who had a chronic history of OH. Acute treatment was effective, but AAG recurred repeatedly. Additionally, it was difficult to judge relapse because of the residual sequelae. During the course, cerebral hemorrhage due to supine hypertension or short-time blood pressure variability and femoral neck fracture caused by OH occurred. She eventually became a wheelchair. This report is clinically important because there are few reports of long-term follow-up of AAG.
摘要：
我们报告了三例抗神经节乙酰胆碱受体（gAChR）抗体阳性的自身免疫性自主神经节病变（AAG）的临床过程，这些病例已经随访了5年以上。在这三种情况下，症状通过急性治疗得到改善，但最终复发了。第一个病例是一名20多岁的女性，她有畏光的慢性病史，便秘和闭经。通过血浆置换，症状几乎消失了，月经恢复了。在课程中,感冒后复发一次。在两次怀孕期间，AAG没有复发。第二例是一名60多岁的男性，他因直立性低血压（OH）和心理症状（婴儿化和心理性假性晕厥）的急性发作而去了医院。虽然IVIg是有效的，它经常复发，难以治疗。然而,所有症状在发病八年后消失，没有任何特殊原因。第三个病例是80多岁的女性，有长期的OH病史。急性治疗是有效的，但AAG反复复发。此外,由于残留的后遗症，很难判断复发。在课程中,仰卧位高血压或短时血压变异性引起的脑出血和OH引起的股骨颈骨折。她最终成了轮椅。该报告在临床上很重要，因为很少有关于AAG长期随访的报告。
