Background. Primary Sj？gren’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) ( , 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. 1. Background Primary Sj？gren’s syndrome (PSS) is a frequent systemic autoimmune disease, characterized by mononuclear infiltration of salivary and lachrymal glands, leading to xerostomia and xerophthalmia (sicca syndrome). Neurological manifestations are observed in 8.5？42% of cases [1]. Delalande et al. reported central nervous system involvement in 56 out of 82 patients (68%) with PSS and neurological manifestations [2]. Cognitive manifestations have been observed in 10–50% of PSS patients [3–8]. Few studies have described the cognitive pattern of PSS [4, 5]. This study examined PSS patients and compared the cognitive pattern and the severity of cognitive impairment to those seen in patients with multiple sclerosis (MS) and the correlations with brain lesions on magnetic resonance imaging (MRI). 2. Patients and Methods Twenty-five consecutive patients (mean age 55 years, range 30–74, 21 women and 4 men) with PSS diagnosed according to American-European Consensus Group criteria [9] were evaluated prospectively at the Neuropsychology unit of the University Hospital of Strasbourg from January 2008 to April 2012. Six patients were diagnosed for PSS at the Neuropsychology unit the others were diagnosed for PSS previously, because of rheumatological and/or
References
[1]
J. De Seze, S. Delalande, and P. Vermersch, “Neurological manifestations in Sjogren syndrome,” La Revue de Médecine Interne, vol. 26, no. 8, pp. 624–636, 2005.
[2]
S. Delalande, J. De Seze, A.-L. Fauchais et al., “Neurologic manifestations in primary Sjogren syndrome: a study of 82 patients,” Medicine, vol. 83, no. 5, pp. 280–291, 2004.
[3]
M. Mataró, D. Escudero, M. Ariza et al., “Magnetic resonance abnormalities associated with cognitive dysfunction in primary Sjogren Syndrome,” Journal of Neurology, vol. 250, no. 9, pp. 1070–1076, 2003.
[4]
K. L. Malinow, R. Molina, and B. Gordon, “Neuropsychiatric dysfunction in primary Sjogren's syndrome,” Annals of Internal Medicine, vol. 103, no. 3, pp. 344–350, 1985.
[5]
C. Lafitte, Z. Amoura, P. Cacoub et al., “Neurological complications of primary Sjogren's syndrome,” Journal of Neurology, vol. 248, no. 7, pp. 577–584, 2001.
[6]
E. Harboe, A. B. Tjensvoll, S. Maroni et al., “Neuropsychiatric syndromes in patients with systemic lupus erythematosus and primary Sjogren syndrome: a comparative population-based study,” Annals of the Rheumatic Diseases, vol. 68, no. 10, pp. 1541–1546, 2009.
[7]
V. Le Guern, C. Belin, C. Henegar et al., “Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with primary Sjogren syndrome: a case-control study,” Annals of the Rheumatic Diseases, vol. 69, no. 1, pp. 132–137, 2010.
[8]
B. M. Segal, B. Pogatchnik, E. Holker et al., “Primary Sjogren's syndrome: cognitive symptoms, mood, and cognitive performance,” Acta Neurologica Scandinavica, vol. 125, no. 4, pp. 272–278, 2012.
[9]
C. Vitali, S. Bombardieri, R. Jonsson et al., “Classification criteria for Sjogren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group,” Annals of the Rheumatic Diseases, vol. 61, no. 6, pp. 554–558, 2002.
[10]
K. Dujardin, P. Sockeel, M. Cabaret, J. De Sèze, and P. Vermersch, “BCcogSEP: a French test battery evaluating cognitive functions in multiple sclerosis,” Revue Neurologique, vol. 160, no. 1, pp. 51–62, 2004.
[11]
W. I. McDonald, A. Compston, G. Edan et al., “Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the Diagnosis of Multiple Sclerosis,” Annals of Neurology, vol. 50, no. 1, pp. 121–127, 2001.
[12]
S. M. Rao, G. J. Leo, L. Bernardin, and F. Unverzagt, “Cognitive dysfunction in multiple sclerosis—I. Frequency, patterns, and prediction,” Neurology, vol. 41, no. 5, pp. 685–691, 1991.
[13]
R. Midgard, T. Riise, and H. Nyland, “Impairment, disability and handicap in multiple sclerosis: a cross-sectional study in an incident cohort in M？re and Romsdal County, Norway,” Journal of Neurology, vol. 243, no. 4, pp. 337–344, 1996.
[14]
J. F. Kurtzke, “Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS),” Neurology, vol. 33, no. 11, pp. 1444–1452, 1983.
[15]
R. C. Petersen, “Mild cognitive impairment as a diagnostic entity,” Journal of Internal Medicine, vol. 256, no. 3, pp. 183–194, 2004.
[16]
B. Dubois, H. H. Feldman, C. Jacova et al., “Research criteria for the diagnosis of Alzheimer's disease: revising the NINCDS-ADRDA criteria,” Lancet Neurology, vol. 6, no. 8, pp. 734–746, 2007.
[17]
I. G. McKeith, D. W. Dickson, J. Lowe, et al., “Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium,” Neurology, vol. 65, pp. 1863–1872, 2005.
[18]
K. Rascovsky, J. R. Hodges, D. Knopman et al., “Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia,” Brain, vol. 134, no. 9, pp. 2456–2477, 2011.
[19]
D. Wechsler, Wechsler Adult Intelligence Scale-Revised Manual, The Psychological Corporation, New York, NY, USA, 1981.
[20]
A. T. Beck, C. H. Ward, M. Mendelson, J. Mock, and J. Erbaugh, “An inventory for measuring depression,” Archives of General Psychiatry, vol. 4, pp. 561–571, 1961.
[21]
L. O. Wahlund, F. Barkhof, F. Fazekas et al., “A new rating scale for age-related white matter changes applicable to MRI and CT,” Stroke, vol. 32, no. 6, pp. 1318–1322, 2001.
[22]
F. Blanc, M. Fleury, A.-S. Korganow et al., “Cognitive and behavioral disorders associated with Gougerot-Sjogren's syndrome,” Revue Neurologique, vol. 165, no. 4, pp. F267–F272, 2009.
[23]
E. Harboe, M. K. Beyer, O. J. Greve et al., “Cerebral white matter hyperintensities are not increased in patients with primary Sjogren's syndrome,” European Journal of Neurology, vol. 16, no. 5, pp. 576–581, 2009.
[24]
A.-L. Fauchais, B. Ouattara, G. Gondran et al., “Articular manifestations in primary Sjogren's syndrome: clinical significance and prognosis of 188 patients,” Rheumatology, vol. 49, no. 6, pp. 1164–1172, 2010.
[25]
B. M. Segal, B. A. Mueller, X. Zhu et al., “Disruption of brain white matter microstructure in primary Sjogren's syndrome: evidence from diffusion tensor imaging,” Rheumatology, vol. 49, no. 8, pp. 1530–1539, 2010.
[26]
A. Creange, D. Laplane, K. Habib, N. Attal, and V. Assuerus, “Dementia disclosing primary Gougerot-Sjogren syndrome,” Revue Neurologique, vol. 148, no. 5, pp. 376–380, 1992.
[27]
J. L. Cummings, “Subcortical dementia. Neuropsychology, neuropsychiatry, and pathophysiology,” British Journal of Psychiatry, vol. 149, pp. 682–697, 1986.
[28]
S. S. Kassan and H. M. Moutsopoulos, “Clinical manifestations and early diagnosis of Sjogren syndrome,” Archives of Internal Medicine, vol. 164, no. 12, pp. 1275–1284, 2004.
