破碎红纤维在神经肌肉疾病中的意义。

The significance of ragged-red fibres in neuromuscular disease.

作者信息

Swash M, Schwartz M S, Sargeant M K

出版信息

J Neurol Sci. 1978 Oct;38(3):347-55. doi: 10.1016/0022-510x(78)90141-7.

Abstract

The pathological significance of ragged-red fibres is uncertain. We have studied ragged-red fibres in the muscle biopsies of 3 adults; one with polymyositis and two with progressive external ophthalmoplegia. All the ragged-red fibres were Type 1 fibres. In two patients the mean diameter of the ragged-red fibres was significantly smaller than the unaffected Type 1 fibres. Some of these fibres showed features of regeneration, and others of degeneration. In the patient with polymyositis the mitochondria were proliferated and contained osmiophilic dense bodies; in the other two patients paracrystalline mitochondrial inclusions were prominent. These findings suggest that ragged-red fibres do not represent a single pathological process.

摘要

破碎红纤维的病理意义尚不确定。我们研究了3名成年人肌肉活检中的破碎红纤维；1例患有多发性肌炎，2例患有进行性眼外肌麻痹。所有破碎红纤维均为1型纤维。在两名患者中，破碎红纤维的平均直径明显小于未受影响的1型纤维。其中一些纤维表现出再生特征，另一些则表现出退变特征。在患有多发性肌炎的患者中，线粒体增殖并含有嗜锇致密小体；在另外两名患者中，线粒体副结晶包涵体突出。这些发现表明，破碎红纤维并不代表单一的病理过程。