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[急性全自主神经功能不全及急性自主神经与感觉神经病]

[Acute pandysautonomia and acute autonomic and sensory neuropathy].

作者信息

Okada F

机构信息

Health Administration Center, Hokkaido University, Sapporo, Japan.

出版信息

Hokkaido Igaku Zasshi. 1989 Jan;64(1):4-9.

PMID:2656462
Abstract

Acute pandysautonomia and acute autonomic and sensory neuropathy were reviewed with special reference to their clinical entity, pathogenesis and clinical course. Since acute pandysautonomia was primarily described as an entity by Young et al. in 1969, a number of similar cases have been described. The disorder is characterized by severe sympathetic and parasympathetic impairment with relative or complete preservation of somatic motor and sensory functions. Some cases have only shown a cholinergic dysautonomia, while others have displayed a loss of autonomic function together with other impairments of nervous function. In 1980, Colan et al. reported a patient with acute autonomic and sensory neuropathy, manifesting severe sensory impairment and dysautonomia with marked loss of myelinated and unmyelinated fibers. Several other similar cases have appeared in the literature. Four years prior to the Colan et al. report, the author described a case which showed almost the same symptoms. At the present time, it is not clear whether this disorder is a new syndrome that is different from acute pandysautonomia or merely a subtype of it. The causes of the above two syndromes are unknown; however, an immunological disorder similar to the Guillain-Barré syndrome has been suggested. The clinical course is often protracted with slow improvement. However, from the author's experience, a relatively rapid improvement occurs after a single systemic administration of either parasympathomimetic or sympathomimetic agonists. Acute pandysautonomia which includes acute autonomic and sensory neuropathy has recently become more common: A number of similar cases have been reported worldwide, including many areas in Japan.

摘要

对急性全自主神经功能不全以及急性自主神经与感觉神经病进行了综述,特别涉及其临床特征、发病机制和临床病程。自1969年扬等人首次将急性全自主神经功能不全描述为一种疾病实体以来,已报道了许多类似病例。该疾病的特征是严重的交感和副交感神经功能障碍,而躯体运动和感觉功能相对保留或完全保留。一些病例仅表现为胆碱能自主神经功能障碍,而另一些病例则表现为自主神经功能丧失以及其他神经功能损害。1980年,科兰等人报告了一例急性自主神经与感觉神经病患者,表现为严重的感觉障碍和自主神经功能障碍,伴有明显的有髓和无髓纤维丧失。文献中还出现了其他几例类似病例。在科兰等人报告的四年前,作者描述了一例表现出几乎相同症状的病例。目前尚不清楚这种疾病是一种不同于急性全自主神经功能不全的新综合征,还是仅仅是其一个亚型。上述两种综合征的病因尚不清楚;然而,有人提出了一种类似于格林-巴利综合征的免疫紊乱。临床病程通常迁延,恢复缓慢。然而,根据作者的经验,单次全身给予拟副交感神经药或拟交感神经药激动剂后会出现相对较快的恢复。包括急性自主神经与感觉神经病在内的急性全自主神经功能不全最近变得更加常见:在世界范围内,包括日本的许多地区,都报道了许多类似病例。

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