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March 28, 2000; 54 (6) Clinical/Scientific Notes

Steroid-responsive Devic’s variant in Sjögren’s syndrome

Akihide Mochizuki, Akito Hayashi, Shin Hisahara, Shin’ichi Shoji
First published March 28, 2000, DOI: https://doi.org/10.1212/WNL.54.6.1391
Akihide Mochizuki
MD
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Akito Hayashi
MD
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Shin Hisahara
MD
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Shin’ichi Shoji
MD
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Citation
Steroid-responsive Devic’s variant in Sjögren’s syndrome
Akihide Mochizuki, Akito Hayashi, Shin Hisahara, Shin’ichi Shoji
Neurology Mar 2000, 54 (6) 1391-1392; DOI: 10.1212/WNL.54.6.1391

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Sjögren’s syndrome is a chronic autoimmune disorder characterized by mononuclear infiltrates in the exocrine glands and various complications involving the peripheral and central nervous systems.1 Optic neuropathy is associated with this disease far less often than it is with other autoimmune vasculitic disorders such as systemic lupus erythematosus (SLE) and giant cell arteritis,2,3 and its mechanism has not been determined. We describe a patient with serologic and biopsy-confirmed Sjögren’s syndrome who had both a corticosteroid-responsive myelopathy and later an optic neuropathy. Additionally, a brain CT showed a swollen intraorbital optic nerve.

Case report.

At 58 years of age, a woman was admitted to the hospital because of the rapid onset of a visual field defect in her left eye. She had a history of a dry sensation in her mouth from her childhood. At age 51, she developed weakness in her arms and legs, had difficulty in walking, and was first admitted to the hospital. Neurologic examination on admission showed tetraparesis, sensory disturbance below the Th6 …

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