A case of reversible cerebral vasoconstriction syndrome without thunderclap headache associated with primary Sjögren syndrome

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  • 原発性シェーグレン症候群に合併した雷鳴頭痛を伴わないreversible cerebral vasoconstriction syndrome の1 例

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A 76-year-old female was presented to our hospital for consciousness disturbance and severe right hemiparesis that was developed one day before admission during the treatment of urinary infection. Any headache did not precede her neurological symptoms. Electroencephalogram revealed marked low activities in the wide area of the left cerebral hemisphere. Brain MRI revealed high intensity lesions in the left temporo-occipital lobes on diffusion weighted images. MR angiography revealed multiple stenosis on the left middle cerebral artery (MCA) and its branches. Cerebral ischemic injury by stenosis of the left MCA was diagnosed, and intravenous administration of argatroban was started. CT angiography taken the next day revealed the recovery of multiple stenotic lesions, and MR angiography taken 22 days after onset revealed no recurrence of arterial stenosis. Her cerebral ischemia was diagnosed as a reversible cerebral vasoconstriction syndrome (RCVS). She gradually improved and attained complete recovery. She had been suffering from dry mouth since 1 year prior to onset, and serum anti-SS-A antibody was positive. Labial salivary gland biopsy performed 40 days after the onset of RCVS revealed lymphocyte infiltration with foci of aggregation of >50 lymphocytes which were dominated by CD3(+) T-cells. Primary Sjögren syndrome associated with central nervous system (CNS) involvement was diagnosed. The pathophysiology underlying CNS in primary Sjögren syndrome includes the direct infiltration of mononuclear cells, vascular involvement, and small vessel vasculitis. Our case suggests that RCVS can be one of the pathogenic mechanisms for CNS involvement in the primary Sjögren syndrome. Argatroban has been shown to prevent cerebral vasospasm and may have therapeutic potential for RCVS.

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