Torbergsen T, Stålberg E, Aasly J, Lindal S
Department of Neurology, University Hospital Tromsö, Norway.
Acta Neurol Scand. 1991 Aug;84(2):132-8. doi: 10.1111/j.1600-0404.1991.tb04921.x.
Electrophysiological studies were performed in 7 patients with Marinesco-Sjögren syndrome in order to search for neuromuscular involvement in this multiorgan disorder. In 6 patients muscle biopsies were also obtained. Light microscopic examinations of the biopsies showed extensive myopathic changes, and in two patients ragged red fibers were found. Electron microscopy showed subsarcolemmal accumulation of abnormal mitochondria in all. Concentric needle EMG revealed unequivocal myopathic changes, more extensive in the anterior tibial than in the biceps brachii muscle. Motor and sensory conduction velocities in the peripheral nerves were normal. There were remarkably high amplitudes of sensory responses. Macro EMG studies in the biceps brachii muscle in four patients showed increased amplitude and area of the macro MUPs. This may be due to abnormal membrane function. Both electrophysiological and morphological findings confirm myopathic features of Marinesco-Sjögren syndrome.
对7例 Marinesco-Sjögren 综合征患者进行了电生理研究，以探寻这种多器官疾病中的神经肌肉受累情况。6例患者还进行了肌肉活检。活检组织的光镜检查显示广泛的肌病性改变，2例患者发现了破碎红纤维。电镜检查显示所有患者肌膜下均有异常线粒体聚集。同心针电极肌电图显示明确的肌病性改变，胫骨前肌比肱二头肌更广泛。周围神经的运动和感觉传导速度正常。感觉反应的波幅显著增高。对4例患者肱二头肌进行的巨肌电图研究显示巨运动单位电位的波幅和面积增加。这可能是由于膜功能异常所致。电生理和形态学检查结果均证实了Marinesco-Sjögren综合征的肌病特征。
