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Case Reports
. 2021 Oct 19;15(1):526.
doi: 10.1186/s13256-021-03103-x.

Cerebellar degeneration in primary Sjögren syndrome: a case report

Seow Lin Chuah  1 , Ahmad Tirmizi Jobli  2 , Sharifah Aishah Wan  1 , Cheng Lay Teh  1
Affiliations
Case Reports

Cerebellar degeneration in primary Sjögren syndrome: a case report

Seow Lin Chuah et al. J Med Case Rep. .

Abstract

Background: Cerebellar degeneration is a rare and severe presentation of primary Sjögren syndrome. There are few case reports of cerebellar degeneration associated with different autoimmune diseases, especially with systemic lupus erythematosus and neuro-Behcet's disease. There are only six patients reported worldwide to be affected by cerebellar atrophy associated with primary Sjögren syndrome. In this report, we describe a patient with primary Sjögren syndrome who presented with ataxia due to cerebellar degeneration.

Case presentation: We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement.

Conclusions: Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.

Keywords: Ataxia; Cerebellar atrophy; Cyclophosphamide; Sjögren syndrome.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Coronal T2WI (left) and sagittal T1WI (right) showing atrophy of the superior and inferior cerebellar vermis (arrow)
See this image and copyright information in PMC

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