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
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- The frequency of sicca syndrome in an elderly female population.J Rheumatol. 1987; 14: 766-771
- Prevalence of Sjögren's syndrome among Nagasaki atomic bomb survivors.Ann Rheum Dis. 2008; 67: 689-695
- Acute transverse myelopathy successfully treated with plasmapheresis and prednisone in a patient with primary Sjögren's syndrome.Arthritis Rheum. 1987; 30: 339-344
- Aseptic meningoencephalitis in primary Gougerot–Sjögren's syndrome.Rev Neurol (Paris). 2005; 161: 1225-1227
- The treatable dementia of Sjögren's syndrome.Ann Neurol. 1991; 30: 99-101
- Chronic inflammatory meningoencephalitis should not be mistaken for Alzheimer's disease.Mayo Clin Proc. 1993; 68: 846-853
- Primary Sjögren's syndrome with subcortical dementia.Intern Med. 1993; 32: 561-564
- Nonvasculitic autoimmune inflammatory meningoencephalitis (NAIM): a reversible form of encephalopathy.Neurology. 1999; 53: 1579-1581
- Nonvasculitic autoimmune inflammatory meningoencephalitis.Neuropathology. 2004; 24: 149-152
- Magnetic resonance imaging of cerebral lesions in patients with the Sjögren syndrome.Ann Intern Med. Jun. 1988; 108: 815-823
- Cerebral white matter lesions in primary Sjögren's syndrome: a controlled study.J Rheumatol. 1999; 26: 1301-1305
- 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
- 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
- Vascular dementia: diagnostic criteria for research studies. Report of the NINDS-AIREN International Workshop.Neurology. 1993; 43: 250-260
- Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium.Neurology. 2005; 65: 1863-1872
- Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria.Neurology. 1998; 51: 1546-1554
- The Dutch normal-pressure hydrocephalus study. How to select patients for shunting? An analysis of four diagnostic criteria.Surg Neurol. 2000; 53: 201-207
- Current concepts in mild cognitive impairment.Arch Neurol. 2001; 58: 1985-1992
- Vascular cognitive disorder: a new diagnostic category updating vascular cognitive impairment and vascular dementia.J Neurol Sci. 2004; 226: 81-87
- 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
- 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
- Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients.Medicine (Baltimore). 2004; 83: 280-291
- Changing perspectives regarding late-life dementia.Nat Rev Neurol. 2009; 5: 649-658
- Is vascular cognitive impairment a useful concept?.J Neurol Sci. 2010; 299: 2-4
- Cerebral small vessel disease: from pathogenesis and clinical characteristics to therapeutic challenges.Lancet Neurol. 2010; 9: 689-701
- Magnetic resonance abnormalities associated with cognitive dysfunction in primary Sjögren syndrome.J Neurol. 2003; 250: 1070-1076
- 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
- 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
- Is there progressive cognitive dysfunction in Sjögren syndrome? A preliminary study.Acta Neurol Scand. 2010; 122: 182-188
Article info
Publication history
Published online: August 20, 2012
Accepted:
July 26,
2012
Received in revised form:
July 25,
2012
Received:
January 28,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.