Objective: To present a rare case of gradually progressing cerebellar ataxia due to systemic autoimmune disease.

Background: Primary Sjögren’s syndrome (pSS) is an autoimmune disease, characterized by lymphocytic infiltration of exocrine glands such as salivary and lacrimal glands leading to xerostomia and xerophthalmia. Neurological manifestations in pSS are diverse and can involve both the central and peripheral nervous systems. Cerebellar ataxia is a rare but potentially debilitating manifestation of pSS, with limited reported cases in the literature (1), (2).

Method: Case report.

Results: A 56-year-old female presented with progressive gait instability and over 9 months. Neurological examination revealed ataxic gait with wide-based, limb incoordination, scanning speech, saccadic hypermetria, and rotational nystagmus consistent with cerebellar dysfunction. She experienced concurrent symptoms of dry eyes and dry mouth. Basic laboratory tests, including complete blood count, metabolic panel, lumbar puncture, and electromyography were within normal limits. Magnetic resonance imaging (MRI) of the brain demonstrated significant atrophy of the vermis of the cerebellum. Further studies showed positive anti-SSA and anti-SSB antibodies, combined with abnormal Schirmer’s test, confirming the diagnosis of Sjögren’s syndrome. Other autoimmune and paraneoplastic etiologies were excluded through extensive serological and imaging studies. The patient was treated with methylprednisolone, hydroxychloroquine, and rituximab. Her neurological symptoms improved slightly until her last visit.

Conclusion: Cerebellar ataxia is a rare but important neurological manifestation of pSS. This case highlights the need for clinicians to consider autoimmune etiologies in patients presenting with progressive ataxia, especially systemic autoimmune diseases such as pSS. Early recognition and management of neurological complications in pSS are crucial to prevent irreversible disability and improve patient outcomes.

References: 1. Chuah SL, Jobli AT, Wan SA, Teh CL. Cerebellar degeneration in primary Sjögren syndrome: a case report. Journal of medical case reports. 2021;15(1):526.
2. Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Annals of the rheumatic diseases. 2020;79(1):3-18.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/progressive-cerebellar-ataxia-in-primary-sjogrens-syndrome-a-case-report/