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眼球运动障碍中眼外肌的超微结构研究。II. 38例活检标本的形态学分析。

Ultrastructural studies of extraocular muscles in ocular motility disorders. II. Morphological analysis of 38 biopsies.

作者信息

Berard-Badier M, Pellissier J F, Toga M, Mouillac N, Berard P V

出版信息

Albrecht Von Graefes Arch Klin Exp Ophthalmol. 1978 Nov 8;208(1-3):193-205. doi: 10.1007/BF00406993.

DOI:10.1007/BF00406993
PMID:215049
Abstract

Thirty eight extraocular muscle biopsies obtained from thirty patients (1 normal and 29 affected with various kinds of ocular motility disorders such as strabismus, Duane's syndrome, paralysis), were studied by electron microscopy. Except for the control biopsy considered as normal, they showed: atrophy, disorganization of myofilaments, double Z-disks, rods, curving arrays of myofibrils, concentrically disposed electron-dense sarcotubules, clusters of mitochondria with numerous modifications of their cristae, and subsarcolemmal inclusions, such as dense bodies, laminated bodies, lipofuscin granules and lipid droplets. In addition, subsarcolemmal granulo-fibrillar aggregates, characterized by periodic granulated foci spaced at about 120 nm intervals, were seen in all the biopsies, even in that of the control case. The authors stress the pecularity of the granulo-fibrillar aggregates which, up to now, have never been described in skeletal muscles and the function of which in the extraocular muscles fibers is impossible to define. They point out the difficulty in determining if the motility disturbances may be related to the various changes observed in extraocular muscles of squinting eyes.

摘要

对30例患者(1例正常,29例患有各种眼球运动障碍,如斜视、杜安综合征、麻痹)的38份眼外肌活检标本进行了电子显微镜研究。除了被视为正常的对照活检标本外,其余标本均显示:萎缩、肌丝排列紊乱、双Z线、杆状体、肌原纤维弯曲排列、同心排列的电子致密肌小管、线粒体簇及其嵴的大量改变,以及肌膜下包涵体,如致密体、层状体、脂褐素颗粒和脂滴。此外,在所有活检标本中,甚至在对照病例的标本中,都可见到肌膜下颗粒-纤维状聚集体,其特征为周期性颗粒状病灶,间隔约120nm。作者强调了颗粒-纤维状聚集体的特殊性,到目前为止,在骨骼肌中从未有过描述,其在眼外肌纤维中的功能也无法确定。他们指出,很难确定眼球运动障碍是否与斜视眼眼外肌中观察到的各种变化有关。

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