Primary Sjögren's Syndrome Presenting as Cerebral Venous Thrombosis: A Rare Case

Authors

Mohan Sonu Chandra
Monisha G A
Ravi Kiran M

Publication Date

9-1-2022

Journal

Cureus

Abstract

Sjogren's syndrome is a late-onset, slowly progressing autoimmune disease characterized by the destruction of the exocrine glands by lymphocytic infiltration, resulting in dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). Sjögren's syndrome may be associated with various autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. We report a case of a 34-year-old female who delivered a live baby 20 days ago. She presented in a postictal state after two episodes of tonic-clonic movements of limbs with altered sensorium with a history of headache for seven days. Further evaluation revealed that the subject had a history of multiple abortions and grittiness in her eyes. MRI showed signs of infarction in the left parietal lobe and magnetic resonance venography (MRV) suggested cavernous venous thrombosis. After an unwavering effort to rule out alternate causes, the rare correlation between primary Sjogren's syndrome and cerebral venous thrombosis was considered. Additional investigations were performed, which showed the patient to be positive for Anti SS-A (Ro52), Anti SS-B (La), and anti-centromere antibodies. The patient gradually improved with anti-edema measures and steroids and was discharged by day nine. We present this case to emphasize the neurological manifestation of Sjogren's syndrome, which may present as cerebral venous thrombosis.

Keywords

cerebral venous thrombosis, headache, keratoconjunctivitis sicca, multiple abortions, sjogrens syndrome