Beyond sicca: A rare case of neuro- sjogren’s presenting as recurrent cerebral infarctions and syringomyelia

• Author Details:   
• Soumia M ^*
• Sunita Aggarwal
• Pradeep Kumar
• Ravindra Kumar Saran
• Rahul Yadav
• Dheeraj Sharma
• Anandswarup Chaudhari

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Background: Neurological involvement in primary Sjögren’s syndrome (pSS) is rare but can include serious central nervous system (CNS) manifestations such as recurrent strokes and myelopathy.

Case Presentation: A 27-year-old woman presented with progressive quadriparesis and dysarthria. Six months earlier, she had recovered from a similar episode of limb weakness. Neurological examination revealed spastic quadriparesis and right 12th cranial nerve palsy. Brain MRI showed chronic infarcts in the bilateral fronto-parietal cortex, right occipital lobe, and basal ganglia, with spinal MRI revealing diffuse cord thinning and a holocord syrinx. Carotid artery Doppler was normal. Autoimmune workup revealed ANA (1:320, speckled) and anti-Ro/SSA positivity. Labial gland biopsy confirmed pSS. CNS vasculitis secondary to pSS was diagnosed, and treatment included IV methylprednisolone followed by weekly rituximab.

Conclusion: This case underscores a rare but severe neurological manifestation of pSS with recurrent infarctions and myelopathy, highlighting the importance of early recognition and immunosuppressive treatment in young patients with unexplained CNS disease