title: Clinical manifestations of neurological involvement in primary Sjögren’s syndrome.
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.
authors.affiliation: Institute of Rheumatology, Tokyo Women’s Medical University, 10-22 Kawada-cho, Shinjuku-Ku Tokyo 162-0054, Japan.
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Kawaguchi
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Katsumata
authors.forename: Kae
authors.initials: K
authors.lastname: Takagi
authors.forename: Akiko
authors.initials: A
authors.lastname: Tochimoto
authors.forename: Sayumi
authors.initials: S
authors.lastname: Baba
authors.forename: Yuko
authors.initials: Y
authors.lastname: Okamoto
authors.forename: Yuko
authors.initials: Y
authors.lastname: Ota
authors.forename: Hisashi
authors.initials: H
authors.lastname: Yamanaka
mesh_terms: D000328:Adult; D002493:Central Nervous System Diseases / Q000145:classification / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000145:classification / Q000209:etiology* / Q000503:physiopathology; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000503:physiopathology
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