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ISRN Neurol. 2013 Sep 19;2013:501327. doi: 10.1155/2013/501327. eCollection 2013.

Cognitive Dysfunction and Dementia in Primary Sjögren's Syndrome.

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Neuropsychology Unit, Service of Neurology, University Hospital of Strasbourg, 1 avenue Molière, 67000 Strasbourg, France ; ICube Laboratory and Strasbourg Federation of Translational Medicine (FMTS), University of Strasbourg and CNRS, Strasbourg, France ; Memory Resource and Research Centre (CMRR) from Alsace, University Hospital of Strasbourg, 1 avenue Molière, 67000 Strasbourg, France.



Primary Sjögren's syndrome (PSS) is a frequent systemic autoimmune disease. In this study, we aimed to explore the cognitive impairment and the correlations with brain MRI.


Twenty-five patients (mean age 55 ± 11.8 years, 21 females) with PSS were prospectively selected and tested with a French translation of the Brief Repeatable Battery for Neuropsychological Examination. The results were compared with the scores for 25 matched patients with multiple sclerosis (MS) and 25 controls. Brain lesions were assessed by brain MRI using the Wahlund classification.


Fifteen of the 25 PSS patients (60%) presented with cognitive disorders versus 19/25 MS patients (76%). Five patients had dementia in the PSS group. Speed of information processing, attention, immediate and long-term memory, and executive functions were frequently impaired. The mean duration of cognitive complaints was 5.6 ± 6.1 years, and the mean duration of PSS was 15.8 ± 14.0 years. A trend towards a correlation was found between the severity of cognitive impairment and the degree of white matter lesions (WML) (P = 0.03, rho = 0.43).


Cognitive impairment-mild or dementia-exists in patients with PSS. Further MRI studies are needed to better understand the precise neural basis of cognitive impairment in PSS patients.

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