A Case of Moyamoya Disease Associated with Systemic Lupus Erythematosus

Lee, Sang Mok; Lee, Young Joo; Park, Woon Gyu

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.