Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

Çoban, Arzu; Özyurt, Selen; Meriç, Kaan; Mısırlı, Handan; Tüzün, Erdem; Türkoğlu, Recai

doi:10.2169/internalmedicine.55.6222

Cited by 9 publications

(5 citation statements)

References 21 publications

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“…Furthermore, as anti-Ro/SSA antibodies were identified in the CSF of the patients with SjS with cerebellar degeneration, it is possible that these antibodies might attack Purkinje cells, reducing Purkinje cell functions and activities, and can cause symptoms of neurological degeneration, resulting in cerebellar atrophy. Several reports have shown the presence of serum anti-Ro/SSA antibodies in patients with SjS with limbic encephalitis [11,29]. We demonstrated that Ro52/TRIM21 was expressed in the neurons of the cortex and hippocampus, which might indicate that anti-Ro/SSA antibodies are involved in other CNS diseases in addition to cerebellar degeneration.…”

Section: Discussionmentioning

confidence: 50%

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

et al. 2021

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Background: Neurological disorders have been identified to be a common extraglandular manifestation of Sjogren's syndrome (SjS). Central nervous system (CNS) symptoms appear in about 5% of patients with SjS. However, so far, only a few incidences of cerebellar degeneration have been reported, and the clinical features and pathological mechanisms associated with SjS remain to be unclear. Intramedullary production of anti-Ro/anti-SjS-related antigen A (SSA) has been observed in some patients with SjS patients who have CNS involvement, suggesting the involvement of anti-Ro/SSA antibodies as antineuronal antibodies in previous studies. Methods: We recently treated cerebellar degeneration in a patient with SjS. We analyzed the serum and cerebrospinal fluid (CSF) in order to detect anti-Ro/SSA and anti-La/anti-SjS-related antigen B (SSB) antibodies. We also searched the literature for previous case reports to evaluate the characteristics of cerebellar degeneration in patients with SjS. First, we have studied in mouse brain tissue and examined whether the Ro/SSA (Ro52/tripartite motif protein (TRIM)21) protein was expressed in the cerebellum of mice using immunohistochemistry. Results: Although all patients that we found in the literature review and our patient 1 were positive for anti-Ro/SSA antibodies, some patients were also negative for anti-La/SSB antibodies. Anti-Ro/SSA antibodies were observed in both serum and CSF; however, anti-Ro/ SSA antibodies were negative in the CSF of patients with SjS without CNS involvement. Cerebellar atrophy was observed, and sequelae remained in the majority of patients. Autopsy findings indicated a selective loss of Purkinje cells. Ro52/TRIM21 expression was also detected throughout murine brains, including the hippocampus, cerebral cortex and cerebellum. High Ro52/TRIM21 expression was observed in the Purkinje cells. Conclusions: We described the characteristics of cerebellar degen-eration in patients with SjS and Ro52/TRIM21 expression in the Purkinje cells of murine cerebellar tissue sections. These outcomes indicate that anti-Ro/SSA antibodies were likely responsible for cerebellar degeneration in patients suffering from SjS.

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Section: Discussionmentioning

confidence: 50%

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

et al. 2021

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“…Furthermore, since anti-Ro/SSA antibodies were identi ed in the CSF of the SjS patients with cerebellar degeneration, it is possible that the anti-Ro/SSA antibodies might attack Purkinje cells, reducing Purkinje cell functions and activities, and cause symptoms of neurologic degeneration, resulting in cerebellar atrophy. Several reports have shown the presence of serum anti-Ro/SSA autoantibodies in SjS patients with limbic encephalitis [9,29]. We demonstrated that Ro52/TRIM21 was expressed in the neurons of the cortex and hippocampus, which might indicate that anti-Ro/SSA autoantibodies are involved in other CNS involvements in addition to cerebellar degeneration.…”

Section: Discussionsupporting

confidence: 49%

Anti-Ro/SAA Autoantibodies Might Represent the Antineuronal Antibodies Responsible for Cerebellar Degeneration in Sjögren's Syndrome 

Tetsuka¹,

Suzuki²,

Ogawa³

et al. 2020

Preprint

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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.

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“…Previous studies on seronegative LE included isolated case reports with incomplete immunological studies, e.g. published before the description of antibodies closely associated with LE such as GABA B R antibodies, LE associated with systemic autoimmune disorders or LE resulting from immunological adverse effects of immune check‐point inhibitors .…”

Section: Discussionmentioning

confidence: 99%

Syndrome and outcome of antibody‐negative limbic encephalitis

Graus

Escudero

Oleaga

et al. 2018

Euro J of Neurology

117

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Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

Cited by 9 publications

References 21 publications

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

Anti-Ro/SAA Autoantibodies Might Represent the Antineuronal Antibodies Responsible for Cerebellar Degeneration in Sjögren's Syndrome

Syndrome and outcome of antibody‐negative limbic encephalitis

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Limbic Encephalitis Associated with Sjögren's Syndrome: Report of Three Cases

Cited by 9 publications

References 21 publications

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

Anti-Ro/SSA Antibodies May Be Responsible for Cerebellar Degeneration in Sjogren’s Syndrome

Anti-Ro/SAA&nbsp;Autoantibodies Might Represent the Antineuronal Antibodies Responsible for Cerebellar Degeneration in Sjögren's Syndrome&nbsp;

Syndrome and outcome of antibody‐negative limbic encephalitis

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Anti-Ro/SAA Autoantibodies Might Represent the Antineuronal Antibodies Responsible for Cerebellar Degeneration in Sjögren's Syndrome