Mihori A, Ohno S, Nakayama M, Ono S, Shimizu N
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital.
Rinsho Shinkeigaku. 1998 Jul;38(7):669-72.
Case 1: A 27-year-old man had a fever of 38 degrees C, followed by acute onset of bilateral upper arm pain. Two days later severe muscle weakness in bilateral upper arms appeared and he was admitted to our hospital. On admission, severe atrophy of the left deltoid and mild atrophy of the right deltoid were observed, with severe muscle weakness in bilateral deltoid and mild weakness in other parts of upper extremities. Tendon reflexes were decreased in the upper extremities. Sensation was intact. CSF showed mild pleocytosis. Nerve conduction velocity was normal and electromyography showed mild NMU decrease in upper extremities. Muscle biopsy of the right deltoid one month after the onset was normal. Muscle weakness began to improve 3 months after the onset, with only mild weakness at 10 months. Case 2: A 60-year-old man had acute onset of left shoulder and upper arm pain, followed by muscle atrophy and weakness of the left upper arm. He showed marked atrophy of the left deltoid, moderate atrophy of the left biceps and left scapular region, and severe muscle weakness in the left upper arm. Deep tendon reflexes were absent in the left upper extremity. Sensation was intact. Nerve conduction velocity was normal and electromyography showed marked NMU decrease in the left upper arm. Muscle biopsy of the left biceps 4 months after the onset showed grouped atrophies on HE staining, type 2 fiber atrophies on routine ATPase staining, and many targetoid atrophic fibers on NADH-TR staining. Muscle weakness began to improve slowly 6 months after the onset, but considerable weakness persisted at 10 months. Detailed muscle biopsy findings in neuralgic amyotrophy have not been documented. Muscle biopsy of Case 2 showed marked neurogenic changes compared to Case 1, which may be associated with the difference in clinical course between the two cases.
病例1:一名27岁男性,体温38摄氏度,随后双侧上臂突发疼痛。两天后双侧上臂出现严重肌肉无力,遂入院治疗。入院时,观察到左侧三角肌严重萎缩,右侧三角肌轻度萎缩,双侧三角肌严重肌肉无力,上肢其他部位轻度无力。上肢腱反射减弱。感觉正常。脑脊液显示轻度细胞增多。神经传导速度正常,肌电图显示上肢轻度运动单位减少。发病后1个月对右侧三角肌进行肌肉活检,结果正常。发病3个月后肌肉无力开始改善,10个月时仅有轻度无力。病例2:一名60岁男性,左肩和上臂突发疼痛,随后出现左上臂肌肉萎缩和无力。他表现为左侧三角肌明显萎缩,左侧肱二头肌和肩胛区中度萎缩,左上臂严重肌肉无力。左上肢深腱反射消失。感觉正常。神经传导速度正常,肌电图显示左上臂明显运动单位减少。发病4个月后对左侧肱二头肌进行肌肉活检,苏木精-伊红染色显示成群萎缩,常规三磷酸腺苷酶染色显示2型纤维萎缩,烟酰胺腺嘌呤二核苷酸-四唑还原酶染色显示许多靶样萎缩纤维。发病6个月后肌肉无力开始缓慢改善,但10个月时仍有相当程度的无力。神经痛性肌萎缩的详细肌肉活检结果尚无文献记载。与病例1相比,病例2的肌肉活检显示明显的神经源性改变,这可能与两例临床病程的差异有关。