Recurrent myelitis in a case of IgG4-related respiratory disease
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- M.D. Tsumura Natsumi
- Department of Neurology, Suzuka General Hospital
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- M.D., Ph.D. Itoh Nobuo
- Department of Neurology, Suzuka General Hospital
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- M.D., Ph.D. Ogawa Ai
- Department of Neurology, Yokkaichi Hazu Medical Center
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- M.D. Maki Toshiki
- Department of Neurology, Suzuka General Hospital
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- M.D., Ph.D. Kuzuhara Shigeki
- Graduate School of Health Science, Suzuka University of Medical Science School of Nursing, Suzuka University of Medical Science
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- M.D., Ph.D. Tomimoto Hidekazu
- Department of Neurology, Mie University Graduate School of Medicine
Bibliographic Information
- Other Title
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- IgG4関連疾患に見られた再発性脊髄炎の1例
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Abstract
<p>We report an 80-year-old man with IgG4-related pleuritis who had been treated with a low dose oral steroid for two years and developed recurrent myelitis. He was admitted to our hospital with gradually worsening numbness in the lower body and difficulty in walking due to mild weakness and loss of proprioception in the legs. T2-weighted MR images of the spinal cord showed a high signal intensity lesion, located centrally in the spinal cord at the Th2–4 spine levels. Laboratory data revealed an elevated serum IgG4 level and cerebrospinal fluid protein level. Anti-aquaporin 4 antibody, anti-myelin oligodendrocyte glycoprotein antibody and other autoantibodies were negative. He showed a good response to the administration of steroid pulse therapy with almost resolution of the neurological symptoms and MRI findings. He was followed with the maintenance therapy with a low dose oral steroid. After one year, he developed recurrence of myelitis in the lower end of the medulla oblongata and in the central to dorsal area at the C2 spine level. Each lesion of recurrent myelitis was located within 3 vertebral segments length and improved without focal spinal atrophy. Recently, IgG4-related disease (IgG4-RD)-associated inflammation involving brain parenchyma and spinal cord were reported. Further investigations are needed to elucidate the relationship between IgG4-RD and seronegative recurrent myelitis.</p>
Journal
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- Rinsho Shinkeigaku
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Rinsho Shinkeigaku 61 (12), 839-843, 2021
Societas Neurologica Japonica
