Objective: This study aimed to characterize the pattern of ataxia in Sjogren’s syndrome and also to describe cerebellar ataxia and cerebellar atrophy in some of these patients.

Background: Sjogren’s syndrome is a systemic autoimmune disorder, often associated with peripheral or central neurological symptoms. Ataxia is rarely reported in Sjogren’s syndrome and may be related to peripheral neuropathy and hardly ever to cerebellar involvement.

Method: We enrolled in this study 14 patients with confirmed Sjogren’s syndrome that presented with gait ataxia. Neurological examination, brain magnetic resonance imaging, and electroneuromyography were performed. Herein we classified ataxia in two main groups: sensory ataxia and cerebellar ataxia.

Results: Four patients with Sjogren’s syndrome and gait ataxia displayed cerebellar sings and three of them had cerebellar atrophy on brain imaging. Three patients had both cerebellar and sensory ataxia and one had pure cerebellar ataxia. The other ten patients with Sjogren’s syndrome and gait ataxia presented pure sensory ataxia, predominantly sensory neuronopathy [table1].

Conclusion: We observed that most cases of ataxia in Sjogren’s syndrome had sensory ataxia, in line with peripheral nerve involvement. Our study also showed that cerebellar ataxia and cerebellar atrophy, although unusual, may be observed in Sjogren’s syndrome with chronic course mimicking a degenerative disease. Although immune-mediated pathophysiological mechanisms are postulated, only one patient with cerebellar ataxia and Sjogren’s syndrome improved after immunotherapy.

References: 1. Chai J, Logigian EL. Neurological manifestations of primary Sjogren’s syndrome. Curr Opin Neurol. 2010;23:509. 2. Barsottini OGP, Albuquerque MVC, Braga-Neto P, Pedroso JL. Adult onset sporadic ataxias: a diagnostic challenge. Arq Neuropsiquiatr. 2014;72:232–40. 3. Maciel R, Camargos S, Cardoso F. Subacute Cerebellar Degeneration as the First Manifestation of Sjögren’s Syndrome. Mov Disord Clin Pract. 2017;4:637–8. 4. Kim MJ, Lee MC, Lee JH, Chung SJ. Cerebellar Degeneration Associated with Sjögren’s Syndrome. J Clin Neurol. 2012;8:155-9. 5. Owada K, Uchihara T, Ishida K, Mizusawa H, Watabiki S, Tsuchiya K. Motor weakness and cerebellar ataxia in Sjögren syndrome—identification of antineuronal antibody: a case report. J Neurol Sci. 2002;197:79-84. 6. Honorat JA, Lopez-Chiriboga AS, Kryzer TJ, Komorowski L, Scharf M, Hinson SR, Lennon VA, Pittock SJ, Klein CJ, McKeon A. Autoimmune gait disturbance accompanying adaptor protein-3B2-IgG. Neurology. 2019;93:e954-e963.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/characterization-of-ataxia-in-sjogrens-syndrome/