Taniguchi A, Niwa A, Nakayama S, Hamaguchi Y, Kuzuhara S
Department of Neurology, Mie University School of Medicine.
Rinsho Shinkeigaku. 1995 Apr;35(4):403-7.
We report a 37-year-old woman who developed chronic thyroiditis, and polymyositis with rimmed vacuoles (RVs). She developed paresthesia of glove and stocking type, muscle weakness of the lower extremities, and slowness in motion. On examination, her lower legs were atrophic and weak, more prominent on the right, while proximal muscles in the upper and lower extremities were slightly affected. Deep tendon reflexes of the upper and lower extremities were normal except for the absent Achilles tendon reflexes. Serum CK was high (1,809 IU/l), and laboratory data indicated chronic thyroiditis and hypothyroidism. Muscle CT showed severe fatty changes in the gastrocnemius and soleus muscles, more marked on the right, and slight atrophy of the thigh muscles. Electromyography and nerve conduction velocity study revealed peripheral neuropathy. Muscle biopsy revealed numerous myofibers with RVs, together with myofiber necrosis, phagocytosis and lymphocyte infiltration, consistent with myositis. Nerve biopsy was indicative of slight axonal degeneration. A diagnosis of polymyositis with RVs in combination with chronic thyroiditis was made. Prednisolone combined with levothyroxine markedly improved muscle weakness though atrophy and weakness of the lower legs were barely improved. Distal-dominant asymmetric affection of the leg muscles and many myofibers with RV on the biopsied muscle are atypical and rare findings of polymyositis. Associated chronic thyroiditis and neuropathy may be related to the formation of marked rimmed-vacuolar degeneration of myofibers in the present case.
我们报告一名37岁女性，她患了慢性甲状腺炎以及伴有镶边空泡（RVs）的多发性肌炎。她出现了手套和袜套样感觉异常、下肢肌肉无力以及运动迟缓。检查发现，她的小腿萎缩且无力，右侧更为明显，而上下肢近端肌肉仅有轻微受累。除跟腱反射消失外，上下肢的深腱反射正常。血清肌酸激酶（CK）升高（1809 IU/l），实验室检查结果提示慢性甲状腺炎和甲状腺功能减退。肌肉CT显示腓肠肌和比目鱼肌有严重的脂肪变性，右侧更为显著，大腿肌肉有轻微萎缩。肌电图和神经传导速度研究显示为周围神经病变。肌肉活检发现大量带有RVs的肌纤维，同时伴有肌纤维坏死、吞噬作用和淋巴细胞浸润，符合肌炎表现。神经活检提示有轻微轴索变性。诊断为伴有RVs的多发性肌炎合并慢性甲状腺炎。泼尼松龙联合左甲状腺素显著改善了肌肉无力，不过小腿的萎缩和无力几乎没有改善。腿部肌肉以远端为主的不对称性受累以及活检肌肉中许多带有RVs的肌纤维是多发性肌炎的非典型且罕见表现。在本病例中，相关的慢性甲状腺炎和神经病变可能与肌纤维显著的镶边空泡变性形成有关。
