Takuma H, Murayama S, Watanabe M, Saito Y, Ugawa Y, Kanazawa I
Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, 113-8655, Tokyo, Japan.
J Neurol Sci. 2000 Apr 15;175(2):140-4. doi: 10.1016/s0022-510x(00)00293-8.
A 46-year-old woman presented progressive proximal weakness and dysphagia. Her serum creatine kinase and myoglobin levels were markedly elevated. Chest X-rays revealed bilateral swelling of the hilar lymph nodes. Needle electromyography demonstrated active denervation and early recruitment. MRI of her skeletal muscle showed focal high intensities on T1-weighted images that were associated with diffusely increased signal intensities on T2-weighted images. Muscle biopsy revealed infiltration of inflammatory cells associated with non-caseating granulomas, and there was widespread segmental fiber necrosis, where necrotic fibers appeared regardless of these granulomas. Immunohistochemical analysis of the surface markers of the infiltrating cells showed CD68- and CD4-positive cells infiltrating into the central area of the granuloma, while CD8-positive cells infiltrating into the endomysium and the periphery of the granulomas. The characteristic histology of the granuloma confirmed the diagnosis of sarcoidosis. The diffuse muscle pathology was consistent with the patient's severe clinical course.
一名46岁女性出现进行性近端肌无力和吞咽困难。她的血清肌酸激酶和肌红蛋白水平显著升高。胸部X线显示双侧肺门淋巴结肿大。针极肌电图显示有活跃的失神经支配和早期募集现象。她骨骼肌的MRI在T1加权图像上显示局灶性高信号，在T2加权图像上伴有弥漫性信号强度增加。肌肉活检显示有与非干酪样肉芽肿相关的炎性细胞浸润，且存在广泛的节段性纤维坏死，坏死纤维的出现与这些肉芽肿无关。对浸润细胞表面标志物的免疫组化分析显示，CD68和CD4阳性细胞浸润到肉芽肿的中心区域，而CD8阳性细胞浸润到肌内膜和肉芽肿的周边。肉芽肿的特征性组织学表现确诊为结节病。弥漫性肌肉病变与患者严重的临床病程相符。
