Lederman R J, Salanga V D, Wilbourn A J, Hanson M R, Dudley A W
Muscle Nerve. 1984 Feb;7(2):142-6. doi: 10.1002/mus.880070210.
We report three patients with inflammatory myopathy who presented clinically with weakness and wasting of only one limb. The myopathy progressed over 6 months and 5 years, respectively, in two patients and was stable after 8 years in the third patient. One patient had a skin rash. Serum CK was elevated in the two patients with progressive disease. Electromyography showed brief duration, small amplitude motor unit potentials and fibrillations in the affected limbs. Muscle biopsy revealed variable fiber size, degenerating and regenerating fibers, and inflammatory foci. Vasculitis was seen in the patient with skin lesions and marked fibroblastic proliferation in the patient with the most chronic course. Immunosuppressive therapy has arrested the progression in the two patients treated; both have regained strength.
我们报告了三名炎性肌病患者,他们临床上仅表现为单肢无力和萎缩。其中两名患者的肌病分别在6个月和5年内进展,第三名患者的病情在8年后稳定。一名患者有皮疹。两名病情进展的患者血清肌酸激酶(CK)升高。肌电图显示受影响肢体的运动单位电位时限短、波幅小以及出现纤颤电位。肌肉活检显示肌纤维大小不一、有纤维变性和再生,以及炎症病灶。有皮肤病变的患者可见血管炎,病程最长的患者有明显的成纤维细胞增殖。免疫抑制治疗已阻止了两名接受治疗患者的病情进展;两人均已恢复力量。