중추 신경계 국한성 맥관염 1례

Abstract

Isolated angiitis of the central nervous system is characterized by necrotizing vascular inflammation confined to the brain or spinal cord. The present report described a 22 year old female patient who experienced abruptly developing headache, dysarthria, swallowing difficulty, quadriparesis, and mental changes. The laboratory test of her blood, urine, and cerebrospinal fluid are all within normal range. Bliateral multiple small areas of low density were noted in the deep brain, cerebellun, and brainstem on brain C-T scan. There is no specific abnormal finding on the cerebral angiogram. The steroid treatment, 3 days of the 'pulse theraphy' followed by oral administration of prednisolone, dramatically ameliorated almost all symptoms and signs at first, but her symptoms were relapsed during tapering of oral prednisolone soon after. The second trial of the steroid treatment was not effective but there was a good response to the combined treatment of cyclophosphamide (Cytoxan) and prednisolone. The repeated brainstem auditary evoked potentials (BAEP) and somatosensory evoked potentials (SSEP) were helpful to determine the clinical course, as well as fluctuating abnormalities on brain CT scans.