Borg K, Edström L
Department of Neurology, Karolinska Hospital, Stockholm, Sweden.
Acta Neurol Scand. 1993 Feb;87(2):128-32. doi: 10.1111/j.1600-0404.1993.tb04091.x.
Patients with prior poliomyelitis with paresis and excessive use (n = 8) and low use (n = 6) of residual anterior tibial motor units (MUs) during walking were subjected to muscle biopsy of the anterior tibial muscle (TA). Antibodies directed against cytoskeletal proteins, spectrin and desmin, and against Leu-19, a myoblast and satellite cell related antigen, were applied. In the patients with excessive use of residual MUs there was an almost total predominance of hypertrophic type I fibres. In the hypertrophic fibres, staining for spectrin and desmin was normal while staining for Leu-19 was seen in a few fibres. Scattered atrophic fibres seen in the patients with excessive use showed staining for spectrin, desmin and Leu-19. In the patients with low use of residual MUs there were extensive pathological muscle fibre changes. Increased staining for spectrin, desmin and Leu-19 was found in most of the atrophic fibres. The predominance of type I fibres in the patients with excessive use of residual MUs is suggested to be due to muscle fibre transformation. The normal staining pattern for spectrin and desmin in the hypertrophic fibres indicates a normal cytoskeletal structure which might suggest that the adaptive muscle fibre changes are adequate to meet the increased demand. The increased staining for spectrin, desmin and Leu-19 in the atrophic fibres might indicate an ongoing denervation process which earlier has been suggested as an important factor for the development of postpolio progressive muscular atrophy.
对既往患有小儿麻痹症且存在轻瘫以及行走时胫前肌运动单位(MU)过度使用(n = 8)和使用不足(n = 6)的患者进行胫前肌(TA)肌肉活检。应用了针对细胞骨架蛋白血影蛋白和结蛋白以及针对成肌细胞和卫星细胞相关抗原Leu-19的抗体。在残余MU过度使用的患者中,几乎完全以I型肥大纤维为主。在肥大纤维中,血影蛋白和结蛋白染色正常,而在少数纤维中可见Leu-19染色。在残余MU过度使用的患者中可见的散在萎缩纤维显示出血影蛋白、结蛋白和Leu-19染色。在残余MU使用不足的患者中存在广泛的病理性肌纤维改变。在大多数萎缩纤维中发现血影蛋白、结蛋白和Leu-19染色增加。残余MU过度使用的患者中I型纤维占优势被认为是由于肌纤维转化。肥大纤维中血影蛋白和结蛋白的正常染色模式表明细胞骨架结构正常,这可能表明适应性肌纤维变化足以满足增加的需求。萎缩纤维中血影蛋白、结蛋白和Leu-19染色增加可能表明正在进行的去神经支配过程,此前这被认为是小儿麻痹后遗症进行性肌肉萎缩发展的一个重要因素。