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Clinical manifestations of neurological involvement in primary Sjögren’s syndrome

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Abstract

The aim of this study was to evaluate neurological manifestations of primary Sjögren’s syndrome (pSS) and investigate the etiology and pathogenesis of peripheral and central nervous complications in pSS. Thirty-two patients with pSS were enrolled in the present study, 20 of whom had neurological involvement plus sicca symptoms. The clinical features were evaluated by neurological examinations including nerve conduction study, magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram. The frequency of fever was significantly higher (P = 0.006) in pSS with neurological involvement than in pSS without neurological involvement. There was no statistical significance in other factors between the two groups. Peripheral nervous system (PNS), central nervous system (CNS), and both PNS and CNS involvements were revealed in 14, 3, and 3 patients, respectively. Optic neuritis and trigeminal neuralgia were revealed frequently in cranial neuropathy. Anti-aquaporin 4 antibody was detected in one patient with optic neuritis. Of the nine patients with polyneuropathy, eight patients presented pure sensory neuropathy including small fiber neuropathy (SFN). pSS with SFN appeared to have no clinically abnormal features, including muscle weakness and decreasing deep tendon reflex. Skin biopsy revealed epidermal nerve fiber degenerated in one pSS patient with pure sensory neuropathy who was diagnosed as having SFN. Our observations suggest that a number of mechanisms can be attributed to neurological involvements in pSS rather than just the mechanisms previously described (i.e., vasculitis and ganglioneuronitis). Presumably, specific autoantibodies may directly induce injury of the nervous system.

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Acknowledgments

We would like to thank Dr. Hiroyuki Nodera for staining of the skin biopsies and Dr. Toshiyuki Takahashi, Yasuto Itoyama, and Keiko Tanaka for detection of the anti-aquaporin 4 antibody. This study was supported by research grants from the Ministry of Health, Labour and Welfare in Japan.

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Authors and Affiliations

  1. Institute of Rheumatology, Tokyo Women’s Medical University, 10-22 Kawada-cho, Shinjuku-Ku, Tokyo, 162-0054, Japan

    Takahisa Gono, Yasushi Kawaguchi, Yasuhiro Katsumata, Kae Takagi, Akiko Tochimoto, Sayumi Baba, Yuko Okamoto, Yuko Ota & Hisashi Yamanaka

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  1. Takahisa Gono
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  2. Yasushi Kawaguchi
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  3. Yasuhiro Katsumata
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  4. Kae Takagi
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  5. Akiko Tochimoto
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  6. Sayumi Baba
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  7. Yuko Okamoto
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  8. Yuko Ota
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  9. Hisashi Yamanaka
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Correspondence to Yasushi Kawaguchi.

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Gono, T., Kawaguchi, Y., Katsumata, Y. et al. Clinical manifestations of neurological involvement in primary Sjögren’s syndrome. Clin Rheumatol 30, 485–490 (2011). https://doi.org/10.1007/s10067-010-1458-7

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  • DOI: https://doi.org/10.1007/s10067-010-1458-7

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