scholarly journals Subacute Cerebellar Degeneration as the First Manifestation of Sjögren's Syndrome

2017 ◽  
Vol 4 (4) ◽  
pp. 637-638 ◽  
Author(s):  
Ricardo Maciel ◽  
Sarah Camargos ◽  
Francisco Cardoso
2021 ◽  
Vol 13 (2) ◽  
pp. 113-120
Author(s):  
Syuichi Tetsuka ◽  
Tomohiro Suzuki ◽  
Tomoko Ogawa ◽  
Ritsuo Hashimoto ◽  
Hiroyuki Kato

2020 ◽  
Author(s):  
Syuichi Tetsuka ◽  
Tomohiro Suzuki ◽  
Tomoko Ogawa ◽  
Ritsuo Hashimoto ◽  
Hiroyuki Kato

Abstract Background: Neurologic disorders are one of the most common extraglandular manifestations of Sjögren's syndrome (SjS). Central neurologic symptoms can appear in about 5% of patients with SjS. However, only a few reports of cerebellar degeneration have been reported, and the clinical features and pathologic mechanisms of cerebellar degeneration associated with SjS are unclear.Patients and methods: We recently treated cerebellar degeneration in a patient with SjS. We analyzed the serum and cerebrospinal fluid (CSF) to detect anti-Ro/anti-SjS-related antigen A (SSA) and anti-La/anti-SjS-related antigen B (SSB) antibodies. We also searched the literature for previous case reports on SjS to evaluate the characteristics of cerebellar degeneration in patients with SjS and examined whether the Ro/SSA (Ro52/tripartite motif protein [TRIM]21) protein was expressed in murine cerebellum using immunohistochemistry.Results: Although all patients were positive for anti-Ro/SSA antibodies, some patients were negative for anti-La/SSB antibodies. Anti-Ro/SSA antibodies were observed in both serum and CSF. Anti-Ro/SSA antibodies were negative in the CSF of SjS patients without central nervous system involvement. Cerebellar atrophy was observed, and sequelae remained in the majority of the patients. Autopsy findings indicated a selective loss of Purkinje cells. Ro52/TRIM21 expression was detected throughout murine brains, including the hippocampus, cerebral cortex, and cerebellum. High Ro52/TRIM21 expression was observed in Purkinje cells.Conclusions: We described the characteristics of cerebellar degeneration in patients with SjS and Ro52/TRIM21 expression in Purkinje cells of murine cerebellar tissue sections. Thus, these outcomes indicated that anti-Ro/SSA autoantibodies were likely responsible for cerebellar degeneration in SjS.


2012 ◽  
Vol 8 (2) ◽  
pp. 155 ◽  
Author(s):  
Mi Jung Kim ◽  
Myoung Chong Lee ◽  
Jae-Hong Lee ◽  
Sun Ju Chung

VASA ◽  
2008 ◽  
Vol 37 (Supplement 73) ◽  
pp. 26-32 ◽  
Author(s):  
Schlattmann ◽  
Höhne ◽  
Plümper ◽  
Heidrich

Background: In order to analyze the prevalence of Raynaud’s syndrome in diseases such as scleroderma and Sjögren’s syndrom – a meta-analysis of published data was performed. Methods: The PubMed data base of the National Library of Medicine was used for studies dealing with Raynaud’s syndrome and scleroderma or Raynaud’s syndroem and Sjögren’s syndrom respectively. The studies found provided data sufficient to estimate the prevalence of Raynaud’s syndrome. The statistical analysis was based on methods for a fixed effects meta-analysis and finite mixture model for proportions. Results: For scleroderma a pooled prevalence of 80.9% and 95% CI (0.78, 0.83) was obtained. A mixture model analysis found four latent classes. We identified a class with a very low prevalence of 11%, weighted with 0.15. On the other hand there is a class with a very high prevalence of 96%. Analysing the association with Sjögren’s syndrome, the pooled analysis leads to a prevalence of Raynaud’s syndrome of 32%, 95% CI(26.7%, 37.7%). A mixture model finds a solution with two latent classes. Here, 38% of the studies show a prevalence of 18.8% whereas 62% observe a prevalence of 38.3%. Conclusion: There is strong variability of studies reporting the prevalence of Raynaud’s syndrome in patients suffering from scleroderma or Sjögren’s syndrome. The available data are insufficient to perform a proper quantitative analysis of the association of Raynaud’s phenomenon with scleroderma or Sjögren’s syndrome. Properly planned and reported epidemiological studies are needed in order to perform a thorough quantitative analysis of risk factors for Raynaud’s syndrome.


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