Cerebellar degeneration in primary Sjogren syndrome: a case report

Background Cerebellar degeneration is a rare and severe presentation of primary Sjogren 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 Sjogren syndrome. In this report, we describe a patient with primary Sjogren syndrome who presented with ataxia due to cerebellar degeneration. Case presentation We report the case of a 37-year-old Chinese woman with primary Sjogren 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 Sjogren 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 Sjogren syndrome. The pathogenesis of the neurological manifestations in primary Sjogren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjogren syndrome with central nervous system involvement. Conclusions Cerebellar degeneration is a rare presentation of primary Sjogren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.

Automated summary

Cerebellar degeneration is a rare presentation of primary Sjogren syndrome, and early diagnosis and treatment are crucial for a good outcome.