A case of overlap syndrome with systemic sclerosis and Sjögren syndrome presenting acute progressive quadriplegia due to hypertrophic spinal pachymeningitis

DOI
  • Chinen Naofumi
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Sato Shinji
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Sasaki Noriko
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Honda Kiri
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Nogi Shinichi
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Saito Eiko
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Yamada Chiho
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Suzuki Yasuo
    Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine
  • Nakamura Naoya
    Department of Pathology, Tokai University School of Medicine

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Other Title
  • 肥厚性脊椎硬膜炎により急速に四肢麻痺が進行した全身性強皮症/シェーグレン症候群オーバーラップの一例

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Abstract

   We report a 61-year-old woman with overlap syndrome with systemic sclerosis(SSc) and Sjögren syndrome(SS) who developed neck pain and progressive quadriplegia. At the time of admission, magnetic resonance imaging(MRI) revealed cervical cord compression by thickened epidural lesions and emergency laminectomy was performed. Epidural specimen showed fibrosis with lymphoid cell infiltration. She was diagnosed as hypertrophic spinal pachymeningitis associated with SSc and SS, and treated with high-dose corticosteroid therapy. Thereafter she showed remarkable improvement in quadriplegia and the cervical thickened epidural lesion on MRI. To the best of our knowledge, this is a rare case of the overlap syndrome with SSc and SS patient complicated with hypertrophic spinal pachymeningitis. In this case, MRI was useful for differentiating hypertrophic spinal pachymeningitis from other conditions responsible for acute progressive quadriplegia.

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