Primary Sjogren's syndrome with central nervous system involvement

ABSTRACT

To describe the clinical, laboratory, and radiological features of Primary Sjogren's syndrome [PSS] with central nervous system [CNS] involvement. A retrospective case series of 12 female patients with PSS and CNS involvement at King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia from 1991-2009. The diagnosis of PSS is defined by the American-European Diagnostic Criteria. We analyzed the clinical, radiological, and immunological features. The mean age was 40 years [range 16-58 years]; all patient were females and presented with active neurological symptoms. The neurological involvement preceded the classic sicca symptoms [33%]. Eight patients [66%] presented with myelopathy, 9 patients [75%] had optic neuritis, and the rest had variable neurological signs. Immunological tests [anti-Sjogren's syndrome A and anti-Sjogren's syndrome B] were high in 7 patients [58%]. Minor salivary gland biopsy revealed inflammatory cell infiltrate in 11 patients [92%]. Brain MRI showed scattered white matter changes in 7 patients [58%]. Spine MRI showed multiple foci of hyperintensity in T2-weighted image in 6 patients [50%], and long segment of hyperintensity at the cervical spinal cord in 2 patients [16%]. Our findings demonstrate that CNS involvements in PSS have great clinical variability and could precede the classic sicca symptoms by years. Primary Sjogren's syndrome can mimic multiple sclerosis [primary progressive multiple sclerosis or relapsing remitting multiple sclerosis], therefore a screening test for PSS should be considered in suspected cases. A well-defined management protocol awaits studies with larger case numbers