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眼肌型重症肌无力:单纤维肌电图的预测价值

Ocular myasthenia gravis: predictive value of single-fiber electromyography.

作者信息

Weinberg D H, Rizzo J F, Hayes M T, Kneeland M D, Kelly J J

机构信息

Department of Neurology, St. Elizabeth's Medical Center and Tufts University School of Medicine, Boston, Massachusetts 02135, USA.

出版信息

Muscle Nerve. 1999 Sep;22(9):1222-7. doi: 10.1002/(sici)1097-4598(199909)22:9<1222::aid-mus8>3.0.co;2-r.

Abstract

Extraocular muscle weakness is the most common presenting sign of myasthenia gravis (MG). More than half of patients presenting with symptoms isolated to these muscles (OMG) develop generalized myasthenia gravis (GMG) over the course of their illness. No clinical, laboratory, or electrophysiological features are recognized that identify these high-risk patients. We have therefore assessed the ability of single-fiber electromyography (SFEMG) to predict the development of GMG in patients presenting with OMG. Thirty-nine consecutive patients presenting with OMG underwent SFEMG of the extensor digitorum communis muscle as well as a battery of other laboratory and imaging studies at the time of diagnosis. All patients were followed prospectively for a minimum of 24 months or until they developed GMG. Two patients were excluded, leaving 37 for assessment. Twenty remained with pure OMG for the entire follow-up period (mean, 55 months). Twenty-six of the 37 had abnormal SFEMG studies at presentation. Eleven of these remained with OMG and 15 developed GMG. Fifty-eight percent of patients with an abnormal SFEMG developed GMG, whereas 82% of those with a normal study remained with OMG. Thus, a normal SFEMG was associated with MG remaining restricted to the extraocular muscles. (P = 0.036, Fisher's exact test), but an abnormal SFEMG was not predictive of subsequent development of GMG.

摘要

眼外肌无力是重症肌无力(MG)最常见的表现症状。超过半数仅出现这些肌肉症状(眼肌型重症肌无力,OMG)的患者在病程中会发展为全身型重症肌无力(GMG)。目前尚未发现能够识别这些高危患者的临床、实验室或电生理特征。因此,我们评估了单纤维肌电图(SFEMG)预测OMG患者发生GMG的能力。39例连续就诊的OMG患者在诊断时接受了指总伸肌的SFEMG检查以及一系列其他实验室和影像学检查。所有患者均进行前瞻性随访,至少随访24个月或直至发展为GMG。两名患者被排除,剩余37例进行评估。20例在整个随访期间(平均55个月)仍为单纯OMG。37例中有26例在初诊时SFEMG检查异常。其中11例仍为OMG,15例发展为GMG。SFEMG检查异常的患者中有58%发展为GMG,而检查正常的患者中有82%仍为OMG。因此,SFEMG正常与MG仅局限于眼外肌相关(P = 0.036,Fisher精确检验),但SFEMG异常并不能预测随后GMG的发生。

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