Document Detail


Primary Sjogren's syndrome with central nervous system involvement.
MedLine Citation:
PMID:  19668890     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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%). CONCLUSION: 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.
Authors:
Iftetah A Alhomoud; Saeed A Bohlega; Mohammed Z Alkawi; Abdulaziz M Alsemari; Saleh M Omer; Fahmi M Alsenani
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Saudi medical journal     Volume:  30     ISSN:  0379-5284     ISO Abbreviation:  Saudi Med J     Publication Date:  2009 Aug 
Date Detail:
Created Date:  2009-08-11     Completed Date:  2009-12-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7909441     Medline TA:  Saudi Med J     Country:  Saudi Arabia    
Other Details:
Languages:  eng     Pagination:  1067-72     Citation Subset:  IM    
Affiliation:
Department of Neurosciences, King Faisal Specialist Hospital & Research Centre, MBC 76, PO Box 3354, Riyadh 11211, Kingdom of Saudi Arabia. Homoudff@yahoo.com
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Central Nervous System Diseases / etiology*
Female
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Retrospective Studies
Sjogren's Syndrome / complications*
Young Adult

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