Kawakami Nobuko, Katsuyama Yusuke, Hagiwara Yuka, Yoshida Hidefumi, Kim Kang, Harada Kiyoshi
Department of Neurology, Shizuoka General Hospital.
Rinsho Shinkeigaku. 2017 Apr 28;57(4):168-173. doi: 10.5692/clinicalneurol.cn-000974. Epub 2017 Mar 30.
A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. Concomitant therapy with prednisolone and azathioprine caused the serum CK level to return to normal and it caused clinical manifestations to abate. One year later, however, muscle weakness worsened. Immunoelectrophoresis of serum revealed IgG M protein, and muscle pathology revealed amyloid deposits in numerous blood vessels and at the periphery of a few muscle fibers, and deposits stained positive for anti-λ light chain antibody. The patient was diagnosed with amyloid myopathy, and therapy for systemic amyloid light chain amyloidosis caused muscle weakness to diminish. Amyloidosis is believed to be the primary pathology in this case based on the patient's response to treatment reaction, but the significance of a case involving both amyloid myopathy and necrotizing myopathy warranted examination.
一名78岁男性出现亚急性进行性近端肌无力和吞咽困难。左股二头肌活检标本显示有坏死和再生肌纤维的证据，但未发现淋巴细胞浸润。该患者被诊断为抗信号识别颗粒（SRP）抗体相关性坏死性肌病。泼尼松龙和硫唑嘌呤联合治疗使血清肌酸激酶（CK）水平恢复正常，并使临床表现减轻。然而，一年后，肌无力加重。血清免疫电泳显示IgG M蛋白，肌肉病理学显示许多血管及少数肌纤维周边有淀粉样沉积物，且沉积物抗λ轻链抗体染色呈阳性。该患者被诊断为淀粉样变性肌病，全身轻链淀粉样变性治疗使肌无力减轻。基于患者对治疗反应，淀粉样变性被认为是本例的主要病理改变，但涉及淀粉样变性肌病和坏死性肌病病例的意义值得探讨。
