A Case of Moyamoya Disease Associated with             Systemic Lupus Erythematosus

J Korean Neurol Assoc > Volume 17(3); 1999 > Article

Journal of the Korean Neurological Association 1999;17(3): 412-415.

전신성 홍반성 낭창과 연관된 모야모야병을 보인 1례

이상목, 이영주, 박운규

한양대학교 의과대학 신경과학교실

A Case of Moyamoya Disease Associated with Systemic Lupus Erythematosus

Sang Mok Lee, M.D., Young Joo Lee, M.D., Woon-Gyu Park, M.D.

Department of Neurology, Hanyang University Kuri Hospital Kyomun-dong 249-1, Kuri-Si, Kyungido, Korea, 471-020

Abstract

Moyamoya disease is a rare clinical entity, diagnosed by cerebral angiography and characterized by occlusion of the bilateral internal carotid artery system and development of collateral arteries. The etiology of moyamoya disease is unknown but some cases were reported to be associated with many other diseases such as hereditary, infectious, autoimmune, and metabolic disorders. A 16-year-old female was admitted with right side motor weakness, visual dis-turbance, malar rash, and emotional lability. Physical examination showed hair loss on frontal and bitemporal areas. Neurologic examinations revealed right hemiparesis and left homonymous hemianopsia. Brain magnetic resonance image showed high signal lesions in the right occipito-temporal and left high frontal area in T2-weighted image. Cerebral angiography showed focal obstructions of supraclinoid portion of both internal carotid arteries and multiple collateral vessels consistent with Moyamoya disease. The clinical features and serologic test of anti-nuclear antibody, anti-double stranded deoxyribonucleic acid antibody and cryoglobulin confirmed the diagnosis of systemic lupus ery-thematosus. We report a case of Moyamoya disease associated with systemic lupus erythematosus which might con-tribute to the etiological variability of Moyamoya disease. J Kor Neurol Ass 17(3):412 ~ 415, 1999 Key Words : Moyamoya disease, Systemic lupus erythematosus, Angiography