Matsuda Nozomu, Sato Shuzo, Nemoto Ayaka, Kubo Hitoshi, Kobayashi Shunsuke, Ugawa Yoshikazu
Department of Neurology, Fukushima Medical University.
Department of Rheumatology, Fukushima Medical University.
Rinsho Shinkeigaku. 2018 Nov 28;58(11):692-695. doi: 10.5692/clinicalneurol.cn-001201. Epub 2018 Oct 27.
A 33-year-old woman developed progressive weakness in the proximal limbs with myalgia and morning stiffness. Physical examination revealed low-grade fever, heliotrope eyelids and mechanic's hand. On neurological examination, she showed Medical Research Council grade 4 weakness in the shoulder girdle, proximal limb muscles, and grade 4 weakness in the abdominis muscle according to Daniels's scale. Laboratory tests revealed elevated serum creatine kinase (6,824 IU/l) and positive anti-PL-7 antibody. A needle electromyography study detected short motor unit potentials of myogenic pattern with abundant fibrillations and positive sharp waves. Whole-body MRI detected high intensity signals in the muscles of the shoulder girdle, proximal limbs, and thoracoabdominal trunk on short-tau inversion recovery sequence images. We diagnosed her as anti-PL-7 myopathy. After treatments with steroid, immunosuppressant, and immunoglobulin, her symptoms improved and abnormal MRI signals were normalized. Although MRI is known to be useful for detection of asymptomatic muscular inflammation in myositis, thoracoabdominal muscles are generally not covered in routine evaluation. To our knowledge, ours is the first case to detect acute inflammation of the thoracoabdominal muscles in antisynthetase syndrome. The present study suggests that whole-body MRI is useful for comprehensive evaluation of muscular involvement and longitudinal assessment for treatment outcomes.
一名33岁女性出现近端肢体进行性无力,伴有肌痛和晨僵。体格检查发现低热、向阳疹眼睑和技工手。神经系统检查显示,根据丹尼尔斯量表,她的肩胛带、近端肢体肌肉医学研究委员会肌力分级为4级,腹肌肌力分级为4级。实验室检查显示血清肌酸激酶升高(6824 IU/l),抗PL - 7抗体阳性。针极肌电图研究检测到肌源性模式的短运动单位电位,伴有大量纤颤电位和正锐波。全身MRI在短tau反转恢复序列图像上检测到肩胛带、近端肢体和胸腹主干肌肉的高强度信号。我们将她诊断为抗PL - 7肌病。经类固醇、免疫抑制剂和免疫球蛋白治疗后,她的症状有所改善,MRI异常信号恢复正常。尽管已知MRI有助于检测肌炎中无症状的肌肉炎症,但胸腹肌肉通常不在常规评估范围内。据我们所知,我们的病例是首例在抗合成酶综合征中检测到胸腹肌肉急性炎症的病例。本研究表明,全身MRI有助于全面评估肌肉受累情况,并对治疗结果进行纵向评估。