SLE and Sjogren's syndrome associated with unilateral moyamoya vessels in cerebral arteries

Moyamoya disease is a rare clinical entity, diagnosed by cerebral angiography and characterized by occlusion of the internal carotid artery system and the development of collateral arteries. A 30-year-old woman with systemic lupus erythematosus and Sjogren's syndrome recurrently presented transient right homonymous hemianopsia. Cerebral angiography showed occlusion of the left posterior cerebral artery associated with the development of collateral circulation (''moyamoya vessels''). In a young adult, as in this case, the unilaterality of the lesion and the presentation of transient ischemic attacks rather than subarachnoid hemorrhage are rare features for Moyamaya disease. Antiphospholipid syndrome was absent.