Takemitsu M, Murayama K, Saga T, Michihiro N, Shiihara H, Kimizuka M, Nonaka I
National Institute of Neuroscience, NCNP, Tokyo, Japan.
Neuromuscul Disord. 1993 Jul;3(4):311-7. doi: 10.1016/0960-8966(93)90024-e.
Two patients with slowly progressive muscle atrophy limited to only one leg are reported. They had pes equinovarus deformity and muscle weakness in the affected leg but no symptom in the other limbs. Muscle biopsies from the affected leg showed dystrophic changes consisting of variation in muscle fiber size, endomysial fibrosis, and necrotic and regenerating fibers. Dystrophin was normally expressed at the surface membrane of the muscle fibers. These two patients possibly had a variant of distal muscular dystrophy, though a neural influence could not be completely excluded.
报告了两名仅局限于一条腿的缓慢进行性肌肉萎缩患者。他们患侧腿部有马蹄内翻足畸形和肌肉无力，但其他肢体无症状。患侧腿部的肌肉活检显示营养不良性改变，包括肌纤维大小变异、肌内膜纤维化以及坏死和再生纤维。肌营养不良蛋白在肌纤维表面膜正常表达。这两名患者可能患有远端型肌营养不良的一种变异型，尽管不能完全排除神经因素的影响。
