进行性肌阵挛性癫痫的病理表现。

Acta neuropathologica. Supplementum Pub Date : 1981-01-01 DOI:10.1007/978-3-642-81553-9_96

S Pierobon-Bormioli, C Angelini, M Armani, G F Testa

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引用次数: 7

摘要

本文对8例进行性肌阵挛性癫痫进行了研究。肌肉活检显示多糖以分支形式积聚，线粒体改变，脂滴和脂褐素体。”“肌肉拉弗拉体”在两例中出现。一种特殊的肌肉细胞骨架外观已经被观察到，这表明新观察到的纤维在它们之间以及与质膜连接的z带存在异常的物理化学状态。

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Myopathological findings in progressive myoclonus epilepsy.

8 patients with progressive myoclonic epilepsy have been studied. Muscle biopsies showed polysaccharide accumulation in branched form, mitochondrial alteration, lipid droplets and lipofucsin bodies. " Muscle Lafora bodies" were present in two cases. A peculiar muscle cytoskeleton appearance has been seen, suggesting an abnormal physico-chemical state of the newly observed filaments that joins Z-bands among them and with the plasma membrane.

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Acta neuropathologica. Supplementum

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