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兰伯特-伊顿肌无力综合征的多样电生理谱。

Diverse electrophysiological spectrum of the Lambert-Eaton myasthenic syndrome.

作者信息

Oh S J

机构信息

Department of Neurology, University of Alabama, Birmingham 35294.

出版信息

Muscle Nerve. 1989 Jun;12(6):464-9. doi: 10.1002/mus.880120605.

Abstract

Among 13 patients with the Lambert-Eaton myathenic syndrome (LEMS), three different patterns on the repetitive nerve stimulation test were observed at the time of initial testing. Type 1 pattern, seen in one patient, had low normal CMAP amplitude, decremental response at the low rate of stimulation (LRS), and relatively normal response at the high rate of stimulation (HRS). Type 2, seen in nine patients, had the classical triad: low CMAP amplitude, decremental response at LRS, and incremental response at HRS. Type 3, seen in three patients, showed low CMAP amplitude, decremental response at LRS, and initial decremental response at HRS. We believe that these three patterns represent different degrees of blocking in LEMS, from the mildest in type 1 to the most severe in type 3. Since types 1 and 3 can be misinterpreted as myasthenia gravis patterns, they must be recognized in LEMS and an incremental response documented by prolonged stimulation at HRS.

摘要

在13例兰伯特-伊顿肌无力综合征(LEMS)患者中,初次检测时在重复神经电刺激试验中观察到三种不同模式。1型模式见于1例患者,复合肌肉动作电位(CMAP)波幅略低于正常,低频刺激(LRS)时反应递减,高频刺激(HRS)时反应相对正常。2型模式见于9例患者,具有典型三联征:CMAP波幅低、LRS时反应递减、HRS时反应递增。3型模式见于3例患者,表现为CMAP波幅低、LRS时反应递减、HRS时最初反应递减。我们认为这三种模式代表了LEMS中不同程度的阻滞,从1型最轻到3型最严重。由于1型和3型可能被误诊为重症肌无力模式,因此在LEMS中必须识别它们,并通过HRS时的延长刺激记录递增反应。

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