Abstract：
Over the past century, generations of neuroscientists, pathologists, and clinicians have elucidated the underlying causes of autonomic failure found in neurodegenerative, inherited, and antibody-mediated autoimmune disorders, each with pathognomonic clinicopathologic features. Autonomic failure affects central autonomic nervous system components in the α-synucleinopathy, multiple system atrophy, characterized clinically by levodopa-unresponsive parkinsonism or cerebellar ataxia, and pathologically by argyrophilic glial cytoplasmic inclusions (GCIs). Two other central neurodegenerative disorders, pure autonomic failure characterized clinically by deficits in norepinephrine synthesis and release from peripheral sympathetic nerve terminals; and Parkinson\'s disease, with early and widespread autonomic deficits independent of the loss of striatal dopamine terminals, both express Lewy pathology. The rare congenital disorder, hereditary sensory, and autonomic neuropathy type III (or Riley-Day, familial dysautonomia) causes life-threatening autonomic failure due to a genetic mutation that results in loss of functioning baroreceptors, effectively separating afferent mechanosensing neurons from the brain. Autoimmune autonomic ganglionopathy caused by autoantibodies targeting ganglionic α3-acetylcholine receptors instead presents with subacute isolated autonomic failure affecting sympathetic, parasympathetic, and enteric nervous system function in various combinations. This chapter is an overview of these major autonomic disorders with an emphasis on their historical background, neuropathological features, etiopathogenesis, diagnosis, and treatment.
摘要：
在过去的一个世纪里,几代神经科学家，病理学家,临床医生已经阐明了神经退行性疾病自主神经功能衰竭的根本原因，继承,和抗体介导的自身免疫性疾病，每个都有病理临床病理特征。自主神经衰竭影响α-突触核蛋白病中的中枢自主神经系统成分，多系统萎缩,临床特征为左旋多巴无反应性帕金森病或小脑共济失调，病理上是嗜银性胶质细胞质内含物（GCI）。另外两种中枢神经退行性疾病，纯自主神经衰竭的临床特征是去甲肾上腺素合成和周围交感神经末端释放的缺陷；和帕金森氏病，早期和广泛的自主神经缺陷独立于纹状体多巴胺末端的损失，都表达路易病理。罕见的先天性疾病,遗传性感官，和III型自主神经病变（或Riley-Day，家族性自主神经失调）由于基因突变而导致危及生命的自主神经衰竭，导致功能压力感受器丧失，有效地将传入机械感应神经元与大脑分离。由靶向神经节α3-乙酰胆碱受体的自身抗体引起的自身免疫性自主神经节病变表现为亚急性孤立的自主神经衰竭，影响交感神经，副交感神经,和肠神经系统功能的各种组合。本章概述了这些主要的自主神经紊乱，重点介绍了它们的历史背景，神经病理学特征，病因，诊断,和治疗。
