2024 Volume 46 Issue 6 Pages 437-442
The patient, a 55-year-old Japanese woman, had been experiencing dry eye since the age of 35 years and subsequently developed an acute pulsatile headache. Brain MRI revealed subcortical frontal lobe hemorrhage and multiple cerebral artery stenosis, which progressively worsened over time. Although a brain biopsy of a lesion in the right frontal lobe did not reveal any conclusive findings suggestive of vasculitis, hematological tests revealed the presence of anti-SS-A antibodies, and a fluorescent dye test confirmed the diagnosis of Sjögren’s syndrome. Based on these findings, the patient was diagnosed with reversible cerebral vasospasm syndrome associated with Sjögren’s syndrome. Her headache gradually disappeared, and the cerebrovascular stenosis improved. There are limited reports on cerebrovascular stenosis in patients with Sjögren’s syndrome, making this case study a valuable contribution to the existing body of knowledge.
