An Autopsy Case of Sjögren's Syndrome with Acute Encephalomyelopathy

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概要

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• A 40-year-old woman developed acute myelopathy and brainstem dysfunction. Magnetic resonance imaging (MRI) revealed high-intensity lesions on T2-weighted axial images (T2WI) in the medulla oblongata and cervical spinal cord. We established a diagnosis of Sjögren's syndrome (SjS) according to the European Community criteria. The patient was treated with methylprednisolone (500 mg/day intravenously) for three days, followed by oral prednisolone. Although her neurological symptoms improved, her general condition deteriorated after the onset of acute colonic pseudo-obstruction and she died of multiple organ failure associated with hemophagocytosis. Autopsy showed atrophy of the secretory glands and an accumulation of lymphocytes around the ducts, confirming the diagnosis of Sjögren's syndrome. Neuropathological examination revealed multifocal lesions in the cervical spinal cord and medulla, along with scattered perivascular lymphocytic infiltration. In addition, there was demyelination, spongy change and axonal swelling in the white matter, but no remarkable vasculitic changes were seen in the central nervous system.

• 日本内科学会の論文

著者

• Houzen Hideki

  Department Of Neurology Obihiro Kosei Hospital

• Houzen Hideki

  Department Of Neurology Obihiro Kosei General Hospital

• YABE Ichiro

  Department of Neurology, Graduate School of Medicine, Hokkaido University

• SASAKI Hidenao

  Department of Neurology, Graduate School of Medicine, Hokkaido University

• KIKUCHI Keisuke

  Department of Pathology, Sapporo Medical University School of Medicine

• Sasaki Hidenao

  Department Of Neurology Graduate School Of Medicine Hokkaido University

• HATA Dai

  Department of Neurology, Obihiro Kosei General Hospital

• Hata Dai

  Department Of Neurology Obihiro Kosei General Hospital

• Yaguchi Hiroaki

  Department of Neurology, Kushiro Rosai Hospital

• Ura Shigehisa

  Department of Neurology, Obihiro Kosei General Hospital

• Takeda Tsuyoshi

  The Third Department of Internal Medicine, Obihiro Kosei General Hospital

• Kikuchi Keisuke

  Department Of Pathology Sapporo Medical University School Of Medicine

• Sasaki Hidenao

  Department Of Internal Medicine Division Of Neurology St. Marianna University School Of Medicine

• Yaguchi Hiroaki

  Department Of Chemistry Faculty Of Science Tokyo University Of Science

• KIKUCHI Keisuke

  Department of Clinical Pathology, Obihiro Kosei General Hospital

• Kikuchi Keisuke

  Department of Pathology, Obihiro Kosei General Hospital

• Yaguchi Hiroaki

  Department of Neurology, Obihiro Kosei General Hospital

• Ura Shigehisa

  Department of Neurology, Asahikawa Red Cross Hospital

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