Goldstein David S, Robertson David, Esler Murray, Straus Stephen E, Eisenhofer Graeme
Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 6N252, 10 Center Drive MSC-1620, Bethesda, MD 20892-1620, USA.
Ann Intern Med. 2002 Nov 5;137(9):753-63. doi: 10.7326/0003-4819-137-9-200211050-00011.
The term dysautonomia refers to a change in autonomic nervous system function that adversely affects health. The changes range from transient, occasional episodes of neurally mediated hypotension to progressive neurodegenerative diseases; from disorders in which altered autonomic function plays a primary pathophysiologic role to disorders in which it worsens an independent pathologic state; and from mechanistically straightforward to mysterious and controversial entities. In chronic autonomic failure (pure autonomic failure, multiple system atrophy, or autonomic failure in Parkinson disease), orthostatic hypotension reflects sympathetic neurocirculatory failure from sympathetic denervation or deranged reflexive regulation of sympathetic outflows. Chronic orthostatic intolerance associated with postural tachycardia can arise from cardiac sympathetic activation after "patchy" autonomic impairment or blood volume depletion or, as highlighted in this discussion, from a primary abnormality that augments delivery of the sympathetic neurotransmitter norepinephrine to its receptors in the heart. Increased sympathetic nerve traffic to the heart and kidneys seems to occur as essential hypertension develops. Acute panic can evoke coronary spasm that is associated with sympathoneural and adrenomedullary excitation. In congestive heart failure, compensatory cardiac sympathetic activation may chronically worsen myocardial function, which rationalizes treatment with beta-adrenoceptor blockers. A high frequency of positive results on tilt-table testing has confirmed an association between the chronic fatigue syndrome and orthostatic intolerance; however, treatment with the salt-retaining steroid fludrocortisone, which is usually beneficial in primary chronic autonomic failure, does not seem to be beneficial in the chronic fatigue syndrome. Dysautonomias are an important subject in clinical neurocardiology.
自主神经功能障碍一词指的是自主神经系统功能的改变，这种改变会对健康产生不利影响。这些改变范围广泛，从神经介导性低血压的短暂、偶发发作到进行性神经退行性疾病；从自主神经功能改变起主要病理生理作用的疾病到自主神经功能改变使独立病理状态恶化的疾病；以及从机制简单明了到神秘且有争议的病症。在慢性自主神经功能衰竭（纯自主神经功能衰竭、多系统萎缩或帕金森病中的自主神经功能衰竭）中，直立性低血压反映了交感神经去神经支配或交感神经输出的反射调节紊乱导致的交感神经循环衰竭。与体位性心动过速相关的慢性直立不耐受可能源于“散在性”自主神经损伤或血容量减少后的心脏交感神经激活，或者，如本讨论中所强调的，源于使交感神经递质去甲肾上腺素向其心脏受体传递增加的原发性异常。随着原发性高血压的发展，心脏和肾脏的交感神经活动似乎会增加。急性惊恐可诱发与交感神经和肾上腺髓质兴奋相关的冠状动脉痉挛。在充血性心力衰竭中，代偿性心脏交感神经激活可能会长期使心肌功能恶化，这使得使用β - 肾上腺素能受体阻滞剂进行治疗具有合理性。倾斜试验阳性结果的高发生率证实了慢性疲劳综合征与直立不耐受之间的关联；然而，通常对原发性慢性自主神经功能衰竭有益的保盐类固醇氟氢可的松治疗，在慢性疲劳综合征中似乎并无益处。自主神经功能障碍是临床神经心脏病学中的一个重要课题。
