Primary Sjogren's syndrome with central nervous system involvement.
OBJECTIVE
To describe the clinical, laboratory, and radiological features of Primary Sjogren's syndrome (PSS) with central nervous system (CNS) involvement.


METHODS
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.


RESULTS
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%).


CONCLUSIONS
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.
Alhomoud
Iftetah A
IA
Department of Neurosciences, King Faisal Specialist Hospital & Research Centre, MBC 76, PO Box 3354, Riyadh 11211, Kingdom of Saudi Arabia. Homoudff@yahoo.com
Bohlega
Saeed A
SA
Alkawi
Mohammed Z
MZ
Alsemari
Abdulaziz M
AM
Omer
Saleh M
SM
Alsenani
Fahmi M
FM
D000293:Adolescent; D000328:Adult; D002493:Central Nervous System Diseases / Q000209:etiology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications*; D055815:Young Adult