Low P A
Department of Neurology, Mayo Clinic, Rochester, MN 55905.
Curr Opin Neurol. 1994 Oct;7(5):402-6. doi: 10.1097/00019052-199410000-00007.
There is a spectrum of acute autonomic neuropathies. Acute panautonomic neuropathy (pandysautonomia), characterized by severe widespread sympathetic and parasympathetic failure, is at one extreme. Guillain-Barré syndrome is at the other end of the spectrum, where the brunt of the disorder falls on the somatic nervous system. Pure acute panautonomic neuropathies are relatively rare. The majority of acute autonomic neuropathies have some minor somatic features. Dysautonomia may be restricted to the cholinergic system (acute cholinergic neuropathy), adrenergic system, or other organ systems (eg, motility disorders). Recently, an important gap in the clinical description of idiopathic autonomic neuropathies and their course was filled, with neurologic and autonomic testing end-points. About one in four to one in three patients will improve substantially. Two other manifestations of autonomic neuropathy are the pseudoobstruction and postural tachycardia syndromes. Loss of the baroreceptors and consequent impairment of buffering of blood pressure results in wild swings in blood pressure that are treatable. Correcting anemia improves orthostatic tolerance, if necessary by hemopoietin. Improved evaluation of neural structure, in skin and in the laboratory, has led to better quantitation of autonomic failure and mechanisms of syncope. This understanding has been extended to the frequency domain and to a study of cerebral vasoregulation.
急性自主神经病变有多种类型。急性全自主神经病变(广泛性自主神经功能不全)处于一个极端,其特征是严重的广泛交感和副交感神经功能衰竭。吉兰 - 巴雷综合征则处于谱系的另一端,该疾病主要影响躯体神经系统。单纯的急性全自主神经病变相对少见。大多数急性自主神经病变有一些轻微的躯体特征。自主神经功能障碍可能局限于胆碱能系统(急性胆碱能神经病)、肾上腺素能系统或其他器官系统(如运动障碍)。最近,随着神经学和自主神经检测终点的出现,特发性自主神经病变及其病程的临床描述中的一个重要空白得以填补。约四分之一至三分之一的患者会有显著改善。自主神经病变的另外两种表现是假性肠梗阻和体位性心动过速综合征。压力感受器丧失以及随之而来的血压缓冲功能受损会导致血压大幅波动,这种情况是可治疗的。如有必要,通过促红细胞生成素纠正贫血可提高直立耐受性。对皮肤和实验室中神经结构评估的改进,使得对自主神经功能衰竭和晕厥机制有了更好的量化。这种认识已扩展到频域以及对脑血管调节的研究。