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伴有缺氧后昏迷的周期性同步性刻板肌阵挛

Periodic synchronous and stereotyped myoclonus with postanoxic coma.

作者信息

Wolf P

出版信息

J Neurol. 1977 Apr 28;215(1):39-47. doi: 10.1007/BF00312548.

Abstract

5 patients with cerebral anoxia of various etiology had postanoxic coma with the syndrome of periodic synchronous and sterotyped myoclonus first mentioned by Lance and Adams. These authors observed action myoclonus as a sequel to this syndrome which is rarely survived. Most reports have dealt mainly with its bad prognostic significance. Although the localization of action myoclonus has much been discussed, and is still controversial, little attention has been paid to the possible localizing significance of its acute precursor syndrome. Most patients with this type of myoclonus (all in our series) present a lower pontine/upper oblongata syndrome. The distribution patterns of the jerks are constant, do not represent topographic but functional relationships showing innervation patterns which are presumably integrated in the vestibular system and upper oblongata reticular formation. Interval histograms show that the jerks, unlike those in subacute sclerosing panencephalitis, are not repeated at regular intervals. Several rhythms might be intermingled, or a rhythmic (physiological?) pacemaker could be modified by proprioception of the jerks. The latter possibility is supported by the observation that EEG discharges in one patient became rhythmic when the jerks ceased. The only other instance of rhythmicity was in a very special observation of continuous seesaw alternations of slow jerks of m. levator palpebrae sup., and tonic contractions of m. corrugator glabellae. Rhythmicity in this case had a 1:2 relation to heart rate indicating the nucleus tractus solitarii region as a possible site of origin of the impulses. Our observations indicate an upper oblongata origin for this type of myoclonus. Autopsies in 3 of our cases revealed widespread severe anoxic damage but nothing to add to the pathological localization.

摘要

5例不同病因的脑缺氧患者出现缺氧后昏迷,并伴有Lance和Adams首次提及的周期性同步和刻板性肌阵挛综合征。这些作者观察到动作性肌阵挛是该综合征的后遗症,很少有患者存活。大多数报告主要讨论了其不良预后意义。尽管动作性肌阵挛的定位已被广泛讨论且仍存在争议,但对其急性前驱综合征可能的定位意义关注较少。大多数这类肌阵挛患者(我们系列中的所有患者)表现为脑桥下部/延髓上部综合征。抽搐的分布模式是恒定的,不代表地形学关系,而是显示出可能整合在前庭系统和延髓上部网状结构中的神经支配模式的功能关系。间隔直方图显示,与亚急性硬化性全脑炎中的抽搐不同,这些抽搐不是以规则的间隔重复出现。可能有几种节律相互交织,或者节律性(生理性?)起搏器可能会被抽搐的本体感觉所改变。后一种可能性得到了以下观察结果的支持:一名患者的脑电图放电在抽搐停止时变得有节律。另一个节律性的例子是在一个非常特殊的观察中,上睑提肌的缓慢抽搐和皱眉肌的强直性收缩持续交替出现。在这种情况下,节律性与心率呈1:2关系,表明孤束核区域可能是冲动的起源部位。我们的观察表明这种类型的肌阵挛起源于延髓上部。我们3例患者的尸检显示广泛严重的缺氧损伤,但对病理定位没有更多补充。

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