Background Prevalence of neurological involvements (NI) in primary Sjögren Syndrome (pSS) varies from 10 to 60% and depends on whether they are screened systematically or only when they are symptomatic.
Objectives The aim of the study was to describe the prevalence, clinical patterns and treatment of NI in pSS.
Methods We performed a retrospective study of patients with pSS (American-European Consensus Group criteria) and followed in an internal medical department over a period of 15 years. Patients with NI were enrolled after excluding other potential causes. We did investigate neurologic systems only when patients present with symptoms.
Results Primary Sjögren Syndrome was diagnosed in 155 patients, 41 had neurological manifestations (26.4%). They were 5 male and 36 female. The mean age at disease onset was 49 +/-13 years. The average delay from NI onset to pSS diagnosis was four months for peripheral nervous system (PNS) and 12 months for central nervous system (CNS). NI revealed the disease in 14 cases (34%). CNS and PNS involvements were respectively found in 27 and 19 cases (six patients had both PNS and CNS). In patients with CNS disorders, headache was the most frequent symptom (n=11). Pyramidal syndrome was found in 9 cases and cerebellar syndrome in one patient. Aseptic meningitis was noted in two cases. Brain MRI was performed in 22 cases and showed abnormalities in 82% of cases. T2 and flair weighted hyperintensities were periventricular (n=11), subcortical (n=5), frontal (n=3) and parietal (n=2). Myelitis was found in one patient.
Patients with PNS showed paresthesia (92%), motor deficit (22%) and decreased tendon reflex (18%). Sensory-motor neuropathy was described in 4 cases, pure sensory neuropathy in 6 cases (including one case of small fiber neuropathy) and motor neuropathy in one patient. Mononeuropathy multiplex was found in 4 cases and polyradiculoneuritis in one patient. Neuromuscular biopsy was performed in five patients and showed vasculitis in two cases. Trigeminal nerve and cochlear nerve were respectively involved in four and one patients. Antinuclear antibodies, anti-SSA and anti-SSB antibodies were respectively positive in 71%, 50% and 28% of cases. NI were treated with corticosteroids and immunosuppressive therapy in respectively 56% and 41% of cases. Outcome was good in 48% of the patients. Comparison of clinical and biological features in patients with and without NI showed no significant differences, only fatigue was significantly associated to NI (45.5% vs 12.9%; p=0.005).
Conclusions In our series, NI prevalence was similar to other groups.
CNS involvements were more frequent than PNS manifestations whereas in other ethnic groups they are less frequent.
Disclosure of Interest None declared