Autoimmune Autonomic Ganglionopathy With Reversible Cognitive Impairment
Autoimmune Autonomic Ganglionopathy With Reversible Cognitive Impairment
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DOI：
10.1001/archneurol.2011.2372
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发表时间：
2012-04-01
影响因子：
--
通讯作者：
Freeman, Roy
中科院分区：
文献类型：
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作者：
Gibbons, Christopher H.;Centi, Justin;Freeman, Roy
背景：自身免疫性自主神经节病（AAG）是一种罕见的抗体介导的自主神经节传递受损疾病，导致严重的自主神经衰竭。尽管治疗了自主神经症状，一些AAG患者仍报告病因不明的认知障碍。目的：探讨AAG患者体位性低血压、抗体滴度与认知功能障碍的关系。设计：前瞻性队列。环境：学术医疗中心。参与者：三名AAG患者在坐位和站位血浆置换周期前后进行神经心理测试。主要观察指标：通过血浆置换前后坐位和站立位相对基线的百分比变化来测量患者对神经心理测试的反应，以确定体位性低血压和抗体滴度对认知的影响。结果：直立性低血压和抗体滴度升高与神经心理障碍独立相关（P < 0.05），特别是在执行功能、持续注意力和工作记忆方面。认知功能障碍得到改善，即使在坐着的血压正常的位置，血浆置换和随之而来的抗体水平降低。结论：可逆性认知障碍与直立性低血压和烟碱乙酰胆碱受体自身抗体升高独立相关，从而扩大了自主神经节病的临床范围，从而为认知障碍提供了另一种可治疗的原因。
Background: Autoimmune autonomic ganglionopathy (AAG) is a rare disorder of antibody-mediated impaired transmission across the autonomic ganglia resulting in severe autonomic failure. Some patients with AAG report cognitive impairment of unclear etiology despite treatment of autonomic symptoms.Objective: To investigate the relationship between orthostatic hypotension, antibody titers, and cognitive impairment in patients with AAG.Design: Prospective cohort.Setting: Academic medical center.Participants: Three patients with AAG underwent neuropsychological testing before and after cycles of plasma exchange in both the seated and standing positions.Main Outcome Measures: Patients' responses to neuropsychological tests were measured by percentage change from baseline in the seated and standing positions before and after plasma exchange to determine the effects of orthostatic hypotension and antibody titers on cognition.Results: Orthostatic hypotension and elevated antibody titer were associated independently with neuropsychological impairment (P < .05), particularly in domains of executive function, sustained attention, and working memory. Cognitive dysfunction improved, even in the seated normotensive position, after plasmapheresis and consequent reduction in antibody levels.Conclusion: Reversible cognitive impairment is independently associated with both orthostatic hypotension and elevated nicotinic acetylcholine receptor autoantibodies, thereby expanding the clinical spectrum of autonomic ganglionopathy and, in so doing, providing an additional treatable cause of cognitive impairment.