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伴有急性感觉运动神经病的自主神经功能障碍。急性自主神经病变的新分类。

Dysautonomia with acute sensory motor neuropathy. A new classification of acute autonomic neuropathy.

作者信息

Yokota T, Hayashi M, Hirashima F, Mitani M, Tanabe H, Tsukagoshi H

机构信息

Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.

出版信息

Arch Neurol. 1994 Oct;51(10):1022-31. doi: 10.1001/archneur.1994.00540220068016.

Abstract

OBJECTIVES

To characterize the dysautonomia associated with acute sensory motor neuropathy and to discuss the classification of acute autonomic neuropathy.

DESIGN

Case series.

METHODS

Sympathetic skin response. Local sweat response to acetylcholine. Norepinephrine infusion test and acetylcholinesterase histochemistry of sural nerve biopsy specimens in addition to making conventional analyses of myelinated and unmyelinated fibers.

RESULTS

In 12 patients with chronic neuropathy, acetylcholinesterase-positive fiber density and plantar sympathetic skin response size were well correlated, but in the two patients with acute autonomic sensory and motor neuropathy, there were discrepancies, acetylcholinesterase-positive fiber density being well preserved and sympathetic skin responses being absent. Histologic and electrophysiologic results indicated primary demyelination of the myelinated fibers. In contrast, previous studies of acute autonomic sensory and motor neuropathy reported dysfunction of the sympathetic postganglionic fibers and axonopathic change in myelinated fibers, poor recovery from dysautonomia.

CONCLUSIONS

Dysautonomia with acute idiopathic neuropathy can be divided into two categories--postganglionic axonopathic and preganglionic demyelinating types of the sympathetic efferent pathways. The recovery from dysautonomia produced by the former lesion is poor, but recovery is better for that produced by the latter lesion.

摘要

目的

描述与急性感觉运动性神经病相关的自主神经功能障碍,并讨论急性自主神经病的分类。

设计

病例系列。

方法

交感皮肤反应、对乙酰胆碱的局部出汗反应、去甲肾上腺素输注试验以及腓肠神经活检标本的乙酰胆碱酯酶组织化学,此外还对有髓和无髓纤维进行常规分析。

结果

在12例慢性神经病患者中,乙酰胆碱酯酶阳性纤维密度与足底交感皮肤反应大小密切相关,但在2例急性自主感觉和运动神经病患者中存在差异,乙酰胆碱酯酶阳性纤维密度保存良好而交感皮肤反应缺失。组织学和电生理结果表明有髓纤维存在原发性脱髓鞘。相比之下,先前关于急性自主感觉和运动神经病的研究报告了交感神经节后纤维功能障碍和有髓纤维的轴索性改变,自主神经功能障碍恢复较差。

结论

急性特发性神经病伴发的自主神经功能障碍可分为两类——交感传出通路的节后轴索性和节前脱髓鞘型。前一种病变导致的自主神经功能障碍恢复较差,而后一种病变导致的恢复较好。

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