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

• Published in: Journal of the neurological sciences 2012-11, Vol.322 (1-2), p.217-221
• Main Authors: Yoshikawa, Kenji, Hatate, Jun, Toratani, Naomi, Sugiura, Shiro, Shimizu, Yoshiomi, Takahash, Tsutomu, Ito, Taiji, Fukunaga, Ryuzo
• Format: Article
• Language: English
• Subjects: Indexing in process
• ISSN: 0022-510X
• DOI: 10.1016/j.jns.2012.07.060

Introduction: Sjoegren'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). Conclusions: 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.