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- DOI 10.18231/j.sajcrr.v.12.i.3.2
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Beyond sicca: A rare case of neuro- sjogren’s presenting as recurrent cerebral infarctions and syringomyelia
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
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How to Cite This Article
Vancouver
M S, Aggarwal S, Kumar P, Saran RK, Yadav R, Sharma D, Chaudhari A. Beyond sicca: A rare case of neuro- sjogren’s presenting as recurrent cerebral infarctions and syringomyelia [Internet]. South Asian J Case Rep Rev. 2025 [cited 2025 Oct 02];12(3):55-58. Available from: https://doi.org/10.18231/j.sajcrr.v.12.i.3.2
APA
M, S., Aggarwal, S., Kumar, P., Saran, R. K., Yadav, R., Sharma, D., Chaudhari, A. (2025). Beyond sicca: A rare case of neuro- sjogren’s presenting as recurrent cerebral infarctions and syringomyelia. South Asian J Case Rep Rev, 12(3), 55-58. https://doi.org/10.18231/j.sajcrr.v.12.i.3.2
MLA
M, Soumia, Aggarwal, Sunita, Kumar, Pradeep, Saran, Ravindra Kumar, Yadav, Rahul, Sharma, Dheeraj, Chaudhari, Anandswarup. "Beyond sicca: A rare case of neuro- sjogren’s presenting as recurrent cerebral infarctions and syringomyelia." South Asian J Case Rep Rev, vol. 12, no. 3, 2025, pp. 55-58. https://doi.org/10.18231/j.sajcrr.v.12.i.3.2
Chicago
M, S., Aggarwal, S., Kumar, P., Saran, R. K., Yadav, R., Sharma, D., Chaudhari, A.. "Beyond sicca: A rare case of neuro- sjogren’s presenting as recurrent cerebral infarctions and syringomyelia." South Asian J Case Rep Rev 12, no. 3 (2025): 55-58. https://doi.org/10.18231/j.sajcrr.v.12.i.3.2