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An Autopsy Case of Sjögren's Syndrome with Acute Encephalomyelopathy

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Title: An Autopsy Case of Sjögren's Syndrome with Acute Encephalomyelopathy
Authors: Yaguchi, Hiroaki Browse this author
Houzen, Hideki Browse this author
Kikuchi, Keisuke Browse this author
Hata, Dai Browse this author
Ura, Shigehisa Browse this author
Takeda, Tsuyoshi Browse this author
Yabe, Ichiro Browse this author →KAKEN DB
Sasaki, Hidenao Browse this author →KAKEN DB
Keywords: Sjögren's syndrome
nonvasculitic autoimmune inflammatory meningoencephalitis
Issue Date: 2008
Publisher: 日本内科学会
Journal Title: Internal Medicine
Volume: 47
Issue: 19
Start Page: 1675
End Page: 1680
Publisher DOI: 10.2169/internalmedicine.47.0207
Abstract: 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.
Type: article (author version)
Appears in Collections:医学院・医学研究院 (Graduate School of Medicine / Faculty of Medicine) > 雑誌発表論文等 (Peer-reviewed Journal Articles, etc)

Submitter: 矢部 一郎

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