A case of neuromyelitis optica spectrum disorder (NMOSD) with Sjögren’s syndrome manifested only brain involvement by preceding parotitis
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- Furukawa Takahiro
- Department of Neurology, Institute of Biomedical Sciences, Tokushima University Graduate School
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- Matsui Naoko
- Department of Neurology, Institute of Biomedical Sciences, Tokushima University Graduate School
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- Tanaka Keiko
- Department of Life Science, Medical Research Institute, Department of Neurology, Kanazawa Medical University Department of Cellular Neurobiology, Brain Research Institute, Niigata University
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- Izumi Yuishin
- Department of Neurology, Institute of Biomedical Sciences, Tokushima University Graduate School
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- Kaji Ryuji
- Department of Neurology, Institute of Biomedical Sciences, Tokushima University Graduate School
Bibliographic Information
- Other Title
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- 耳下腺炎を契機に発症し，脳病変のみを反復したシェーグレン症候群合併視神経脊髄炎関連疾患の1例
Abstract
<p>A 33 year-old woman presented with intentional incontinence, motor aphasia, supranuclear gaze palsy, and spasticity after parotitis. Brain magnetic resonance images (MRI) showed abnormal signaling in long corticospinal tract involving internal capsules and cerebral peduncles, middle cerebellar peduncle, and frontal subcortical white matter lesions. She had a long history of dry eye and mouth. Immunoserological study showed that she was positive for anti-SS-A, aquaporin 4 (AQP4), and AQP5 antibodies. She clinically showed not only Sjögren’s syndrome but also neuromyelitis optica spectrum disorder (NMOSD) without optic neuritis or myelitis. She responded to steroid followed by plasma exchange dramatically. Thereafter, the relapse of brain lesion was once detected while tapering of steroid, but her symptoms have been stable for several years after administration of immunosuppressant. This case suggested that salivary gland inflammation might be associated with the pathogenesis of NMOSD. </p>
Journal
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- Rinsho Shinkeigaku
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Rinsho Shinkeigaku 57 (2), 77-81, 2017
Societas Neurologica Japonica