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. 2013 Sep 19:2013:501327.
doi: 10.1155/2013/501327. eCollection 2013.

Cognitive Dysfunction and Dementia in Primary Sjögren's Syndrome

Frederic Blanc  1 , Nadine Longato, Barbara Jung, Catherine Kleitz, Laure Di Bitonto, Benjamin Cretin, Nicolas Collongues, Christelle Sordet, Marie Fleury, Vincent Poindron, Jacques-Eric Gottenberg, Olivier Anne, Dan Lipsker, Thierry Martin, Jean Sibilia, Jérôme de Seze
Affiliations

Cognitive Dysfunction and Dementia in Primary Sjögren's Syndrome

Frederic Blanc et al. ISRN Neurol. .

Abstract

Background. 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. Methods. 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. Results. 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). Conclusion. 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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Figures

Figure 1
Figure 1
Axial brain FLAIR-magnetic resonance imaging of two female patients with primary Sjögren's syndrome. Patient (a) had three cognitive subtests below the 5th percentile and a Wahlund score of 1 for white matter lesions. Patient (b) had five cognitive subtests below the 5th percentile and a Wahlund score of 2 (beginning confluence of lesions) for white matter lesions.
Figure 2
Figure 2
Scatter plot. Cognitive impairment (number of subtests inferior to the 5th percentile among 14) against white matter lesions load (Wahlund classification from 0 to 3). Spearman rank correlation coefficient was rho = 0.43 (P = 0.03); linear curve adjustment (R 2 = 0,205).
See this image and copyright information in PMC

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