Thoracoabdominal muscle involvement in anti-PL-7 myopathy revealed by whole-body magnetic resonance imaging

  • Matsuda Nozomu
    Department of Neurology, Fukushima Medical University
  • Sato Shuzo
    Department of Rheumatology, Fukushima Medical University
  • Nemoto Ayaka
    Advanced Clinical Research Center, Fukushima Medical University
  • Kubo Hitoshi
    Advanced Clinical Research Center, Fukushima Medical University Preparing Section for New Faculty of Medical Sciences, Fukushima Medical University
  • Kobayashi Shunsuke
    Department of Neurology, Fukushima Medical University
  • Ugawa Yoshikazu
    Department of Neurology, Fukushima Medical University Department of Neuro-Regeneration, Fukushima Medical University Department of Neurology, Aidu Chuo Hospital

Bibliographic Information

Other Title
  • Whole-body MRIにより胸腹部の体幹筋障害を確認した抗PL-7抗体陽性筋炎の1例

Description

<p>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.</p>

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 58 (11), 692-695, 2018

    Societas Neurologica Japonica

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