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Prevalence of Sjögren's syndrome with dementia in a memory clinic

Published:August 20, 2012DOI:https://doi.org/10.1016/j.jns.2012.07.060

      Abstract

      Introduction

      Sjögren's syndrome (SS) is an autoimmune disorder involving the exocrine glands, which affects 1.9–3.0% of the elderly population. Approximately 20% of all patients with SS have CNS involvement, including dementia, as a result of angiitis.

      Aims

      The aim of the study was to clarify the prevalence and impact of SS among patients in a memory clinic.

      Methods

      This study prospectively recruited patients with cognitive dysfunction in a memory clinic from 2007 to 2010. In addition to the examinations for dementia, the patients' levels of anti-SSA and SSB antibodies were measured. Schirmer's test and/or a lip biopsy were added if required. SS was diagnosed based on the American European consensus criteria.

      Results

      Out of 276 cases who completed the examinations, 265 (97/168 males/females, mean age: 77.9, median MMSE score: 23) did not demonstrated cognitive decline. Sixteen (6.3%) and seven (2.7%) patients were positive for anti-SS-A and SS-B antibodies, respectively. Twenty patients (7.5%) were diagnosed with primary SS (mean age: 77.2 years old, median MMSE: 21). Seven of these patients had previously been diagnosed with MCI (VCIND: 5, aMCI: 2), and 13 had been diagnosed with dementia. All had asymmetrical focal hypoperfusion on SPECT, and eighteen had subcortical lesions on MRI. Twelve were treated for dementia (median time: 2.1 years), and their MMSE significantly improved (median MMSE: 26, p=0.0019), while the non-SS subjects' MMSE declined (n=126, median: 22).

      Conclusion

      The patients with SS accounted for 7.5% of those with a cognitive decline as determined at a memory clinic, and are characterized by subcortical white matter lesions and asymmetric hypoperfusion.

      Keywords

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      References

        • Strickland R.W.
        • Tesar J.T.
        • Berne B.H.
        • Hobbs B.R.
        • Lewis D.M.
        • Welton R.C.
        The frequency of sicca syndrome in an elderly female population.
        J Rheumatol. 1987; 14: 766-771
        • Hida A.
        • Akahoshi M.
        • Takagi Y.
        • Ashizawa K.
        • Imaizumi M.
        • Soda M.
        • et al.
        Prevalence of Sjögren's syndrome among Nagasaki atomic bomb survivors.
        Ann Rheum Dis. 2008; 67: 689-695
        • Konttinen Y.T.
        • Kinnunen E.
        • von Bonsdorff M.
        • Lillqvist P.
        • Immonen I.
        • Bergroth V.
        • et al.
        Acute transverse myelopathy successfully treated with plasmapheresis and prednisone in a patient with primary Sjögren's syndrome.
        Arthritis Rheum. 1987; 30: 339-344
        • Moutaouakil F.
        • El Moutawakkil B.
        • El Otmani H.
        • Gam I.
        • Rafai M.A.
        • Slassi I.
        Aseptic meningoencephalitis in primary Gougerot–Sjögren's syndrome.
        Rev Neurol (Paris). 2005; 161: 1225-1227
        • Caselli R.J.
        • Scheithauer B.W.
        • Bowles C.A.
        • Trenerry M.R.
        • Meyer F.B.
        • Smigielski J.S.
        • et al.
        The treatable dementia of Sjögren's syndrome.
        Ann Neurol. 1991; 30: 99-101
        • Caselli R.J.
        • Scheithauer B.W.
        • O'Duffy J.D.
        • Peterson G.C.
        • Westmoreland B.F.
        • Davenport P.A.
        Chronic inflammatory meningoencephalitis should not be mistaken for Alzheimer's disease.
        Mayo Clin Proc. 1993; 68: 846-853
        • Kawashima N.
        • Shindo R.
        • Kohno M.
        Primary Sjögren's syndrome with subcortical dementia.
        Intern Med. 1993; 32: 561-564
        • Caselli R.J.
        • Boeve B.F.
        • Scheithauer B.W.
        • O'Duffy J.D.
        • Hunder G.G.
        Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy.
        Neurology. 1999; 53: 1579-1581
        • Josephs K.A.
        • Rubino F.A.
        • Dickson D.W.
        Nonvasculitic autoimmune inflammatory meningoencephalitis.
        Neuropathology. 2004; 24: 149-152
        • Alexander E.L.
        • Beall S.S.
        • Gordon B.
        • Selnes O.A.
        • Yannakakis G.D.
        • Patronas N.
        • et al.
        Magnetic resonance imaging of cerebral lesions in patients with the Sjögren syndrome.
        Ann Intern Med. Jun. 1988; 108: 815-823
        • Coates T.
        • Slavotinek J.P.
        • Rischmueller M.
        • Schultz D.
        • Anderson C.
        • Dellamelva M.
        • Sage M.R.
        • Gordon T.P.
        Cerebral white matter lesions in primary Sjögren's syndrome: a controlled study.
        J Rheumatol. 1999; 26: 1301-1305
        • Le Guern V.
        • Belin C.
        • Henegar C.
        • Moroni C.
        • Maillet D.
        • Lacau C.
        • et al.
        Cognitive function and 99mTc-ECD brain SPECT are significantly correlated in patients with primary Sjögren's syndrome: a case–control study.
        Ann Rheum Dis. 2010; 69: 132-137
        • McKhann G.
        • Drachman D.
        • Folstein M.
        • Katzman R.
        • Price D.
        • Stadlan E.M.
        Clinical diagnosis of Alzheimer's disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer's Disease.
        Neurology. 1984; 34: 939-944
        • Román G.C.
        • Tatemichi T.K.
        • Erkinjuntti T.
        • Cummings J.L.
        • Masdeu J.C.
        • Garcia J.H.
        • et al.
        Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop.
        Neurology. 1993; 43: 250-260
        • Consortium on DLB
        Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium.
        Neurology. 2005; 65: 1863-1872
        • Neary D.
        • Snowden J.S.
        • Gustafson L.
        • Passant U.
        • Stuss D.
        • Black S.
        • et al.
        Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria.
        Neurology. 1998; 51: 1546-1554
        • Boon A.J.
        • Tans J.T.
        • Delwel E.J.
        • Egeler-Peerdeman S.M.
        • Hanlo P.W.
        • Wurzer H.A.
        • et al.
        The Dutch normal-pressure hydrocephalus study. How to select patients for shunting? An analysis of four diagnostic criteria.
        Surg Neurol. 2000; 53: 201-207
        • Petersen R.C.
        • Doody R.
        • Kurz A.
        • Mohs R.C.
        • Morris J.C.
        • Rabins P.V.
        • et al.
        Current concepts in mild cognitive impairment.
        Arch Neurol. 2001; 58: 1985-1992
        • Román G.C.
        • Sachdev P.
        • Royall D.R.
        • Bullock R.A.
        • Orgogozo J.M.
        • López-Pousa S.
        • et al.
        Vascular cognitive disorder: a new diagnostic category updating vascular cognitive impairment and vascular dementia.
        J Neurol Sci. 2004; 226: 81-87
        • Vitali C.
        • Bombardieri S.
        • Jonsson R.
        • Moutsopoulos H.M.
        • Alexander E.L.
        • Carsons S.E.
        • et al.
        Classification criteria for Sjögren's syndrome: a revised version of the European criteria proposed by the American-European Consensus Group.
        Ann Rheum Dis. 2002; 61: 554-558
      1. Committee drafting guideline for the treatment of dementia, Japanese society of Neurology. A guideline for the treatment of dementia.
        Rinsho Shinkeigaku. 2002; 42: 788-833
        • Delalande S.
        • de Seze J.
        • Fauchais A.L.
        • Hachulla E.
        • Stojkovic T.
        • Ferriby D.
        • et al.
        Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients.
        Medicine (Baltimore). 2004; 83: 280-291
        • Fotuhi M.
        • Hachinski V.
        • Whitehouse P.J.
        Changing perspectives regarding late-life dementia.
        Nat Rev Neurol. 2009; 5: 649-658
        • Korczyn A.D.
        • Vakhapova V.
        Is vascular cognitive impairment a useful concept?.
        J Neurol Sci. 2010; 299: 2-4
        • Pantoni L.
        Cerebral small vessel disease: from pathogenesis and clinical characteristics to therapeutic challenges.
        Lancet Neurol. 2010; 9: 689-701
        • Mataró M.
        • Escudero D.
        • Ariza M.
        • Sánchez-Ojanguren J.
        • Latorre P.
        • Junqué C.
        • et al.
        Magnetic resonance abnormalities associated with cognitive dysfunction in primary Sjögren syndrome.
        J Neurol. 2003; 250: 1070-1076
        • Tzarouchi L.C.
        • Tsifetaki N.
        • Konitsiotis S.
        • Zikou A.
        • Astrakas L.
        • Drosos A.
        • et al.
        CNS involvement in primary Sjogren syndrome: assessment of gray and white matter changes with MRI and voxel-based morphometry.
        AJR Am J Roentgenol. 2011; 197: 1207-1212
        • Chang C.P.
        • Shiau Y.C.
        • Wang J.J.
        • Ho S.T.
        • Kao A.
        Abnormal regional cerebral blood flow on 99mTc ECD brain SPECT in patients with primary Sjögren's syndrome and normal findings on brain magnetic resonance imaging.
        Ann Rheum Dis. 2002; 61: 774-778
        • Martínez S.
        • Cáceres C.
        • Mataró M.
        • Escudero D.
        • Latorre P.
        • Dávalos A.
        Is there progressive cognitive dysfunction in Sjögren syndrome? A preliminary study.
        Acta Neurol Scand. 2010; 122: 182-188