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.

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Pulmonary involvement is also common, affecting ≤50% of patients, but it is generally mild [197]. Sjögren's syndrome affects women up to 9 times more often than men [198,199]. The incidence is estimated to be 40 to 50 per million of the population in Europe, with a prevalence of 2000 to 30,000 per million [200].…”

“…The incidence is estimated to be 40 to 50 per million of the population in Europe, with a prevalence of 2000 to 30,000 per million [200]. Sjögren's syndrome can affect both the PNS and the CNS, and ≤60% or more patients with Sjögren's syndrome will develop neurological symptoms, although it is largely due to cryoglobulins and anti-nuclear antibodies (anti-SSA and anti-SSB) [198]. PNS involvement is characterized by sensory polyneuropathy and mononeuritis multiplex [198].…”

“…Sjögren's syndrome can affect both the PNS and the CNS, and ≤60% or more patients with Sjögren's syndrome will develop neurological symptoms, although it is largely due to cryoglobulins and anti-nuclear antibodies (anti-SSA and anti-SSB) [198]. PNS involvement is characterized by sensory polyneuropathy and mononeuritis multiplex [198]. CNS manifestations occur in 6% to 25% of patients with Sjögren's syndrome, depending on the study and the definitions used, and these include encephalopathy, aseptic meningitis, optic neuritis, and MS-like disease [198,201,202].…”

“…PNS involvement is characterized by sensory polyneuropathy and mononeuritis multiplex [198]. CNS manifestations occur in 6% to 25% of patients with Sjögren's syndrome, depending on the study and the definitions used, and these include encephalopathy, aseptic meningitis, optic neuritis, and MS-like disease [198,201,202]. Involvement of the CNS is more likely in patients with lung involvement [201].…”

Nervous system involvement in Sjögren's syndrome and vasculitis

“…[3] SjS'de nörolojik tutulum kad›nlarda 4-30 kat daha s›kt›r. [4] Klinik pratikte hastalar, dizestezi, yan›c› a¤r›, artralji, miyalji gibi ekstremite a¤r›lar›ndan ve vertigo, yüzde k›zar›kl›k, terleme bozukluklar›, diyare, konstipasyon gibi otonom sinir sistemi semptomlar›ndan flikayet ederler. [4] Sjögren sendromunda sinir sistemi tutulumu ve vaskülit …”