Abstract

1.1.  Background: Central nervous system (CNS) involvement in primary Sjogren Syndrome (pSS) is infrequent and often poses a diagnostic dilemma due to its diverse clinical presentation.

1.2.  Case presentation: A 66-year-old woman presented to our department with a three-month history of progressive weakness in the lower limbs, sensory loss, and sphincter dysfunction. Spine MRI revealed an intramedullary hyperintense signal on T2 weighted images extending longitudinally and transversally from D6 to D12. Auto-immune tests, viral serologies, and metabolic workups yielded negative results. A minor salivary gland biopsy confirmed chronic grade III sialadenitis. Primary Sjogren Syndrome was diagnosed based on the 2016 ACR-EULAR classification criteria. The patient underwent treatment with glucocorticoids and cyclophosphamide, showing mild improvement after six months.

1.3.  Conclusion: This case highlights the significance of considering Sjogren syndrome in the differential diagnosis of progressive myelopathy. Given the severity of spinal involvement in pSS, early diagnosis and aggressive treatment from the disease’s onset are crucial.

Keywords

progressive myelopathy, central nervous system, sicca symptoms, primary sjögren syndrome, cyclophosphamide