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Lupus
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case-report

Moyamoya syndrome associated with systemic lupus erythematosus

HC Jeong

Department of Rheumatology, Chonnam National University Medical School and Hospital, Gwangju, Republic of Korea

YJ Kim

Department of Rheumatology, Chonnam National University Medical School and Hospital, Gwangju, Republic of Korea

W Yoon

Department of Radiology, Chonnam National University Medical School and Hospital, Gwangju, Republic of Korea

SP Joo

Department of Neurosurgery, Chonnam National University Medical School and Hospital, Gwangju, Republic of Korea

SS Lee

Department of Rheumatology, Chonnam National University Medical School and Hospital, Gwangju, Republic of Korea

YW Park

Department of Rheumatology, Chonnam National University Medical School and Hospital, Gwangju, Republic of Korea, parkyw{at}chonnam.ac.kr

Abstract

Moyamoya disease is a rare, progressive cerebrovascular disorder that is characterized by a stenosis or occlusion of the bilateral internal carotid arteries and the development of collateral vessels. Transient ischemic attacks or seizures are the usual presentation of moyamoya disease in children, whereas cerebral hemorrhage is the most common symptom in adults. We report an 18-year-old female patient with active lupus nephritis who presented with the sudden onset of left hemiparesis. Brain magnetic resonance imaging showed acute infarctions in the right basal ganglia and subcortical white matter of the right frontal lobe. Cerebral angiography showed the stenosis of the bilateral internal carotid arteries with rich basal collateral vessels (moyamoya vessels). There was no evidence of atherosclerosis or antiphospholipid syndrome. Glucocorticoid therapy was used to control the systemic lupus erythematosus. Prophylactic bypass surgery was performed to prevent recurrent ischemic attacks. This case report shows that an underlying cerebrovascular lesion of moyamoya vessels in a patient with systemic lupus erythematosus is susceptible to cerebrovascular accidents.

Key Words: angiography • cerebral infarction • ischemic attack • moyamoya syndrome • systemic lupus erythematosus

Lupus, Vol. 17, No. 7, 679-682 (2008)
DOI: 10.1177/0961203307087375
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