조직검사로 확진된 고립성 중추신경 혈관염 1례
A Case of Isolated Angiitis of the Central Nervous System with Histologic Confirmation
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Isolated angiitis of the central nervous system(IACNS) is a rare, often fatal disorder that frequently responds to aggressive immunosuppressive therapy. Recently, IACNS has been increasingly diagnosed by angiography without histologic confirmation. We report a 38-year-old man who presented with headache and seizure. A diagnosis of IACNS was verified by pathologic findings with Langhans-type granulomatous giant cells. His symptoms improved without any immunosuppressive therapy. Brain MRI performed 5 months later showed marked improvement. This is a first tissue biopsy proven IACNS in Korea. We emphasize that the wedge biopsy of cortical and leptomeningeal tissues is a significant clue to diagnose an IACNS and suggest that this patient with favorable outcome is a benign subset of IACNS.
Abstract
Isolated angiitis of the central nervous system(IACNS) is a rare, often fatal disorder that frequently responds to aggressive immunosuppressive therapy. Recently, IACNS has been increasingly diagnosed by angiography without histologic confirmation. We report a 38-year-old man who presented with headache and seizure. A diagnosis of IACNS was verified by pathologic findings with Langhans-type granulomatous giant cells. His symptoms improved without any immunosuppressive therapy. Brain MRI performed 5 months later showed marked improvement. This is a first tissue biopsy proven IACNS in Korea. We emphasize that the wedge biopsy of cortical and leptomeningeal tissues is a significant clue to diagnose an IACNS and suggest that this patient with favorable outcome is a benign subset of IACNS.