According to the past reports, neuropsychiatric manifestations have been seen in 
10-75% of patients with systemic lupus erythematosus and are second only to 
renal involvement as a cause of death. The clinical feature is multiple. And 
cerebrovascular diseases due to systemic lupus erythematosus are detected in 
3-16% of the neuropsychiatric manifestations. Occlusion of the intracranial 
major arteries is less frequently found in other cerebrovascular diseases. And 
central nervous system involvement usually occurs at some intermediate or 
terminal stage of systemic lupus erythematosus, so is rarely regarded as one of 
the initial symptoms. We studied the case of a patient with systemic lupus 
erythematosus with occlusion of the right middle cerebral artery indicated by 
angitis and 'string of beads' appearance of the right internal carotid artery 
indicated by fibromuscular dysplasia. The patient was a 38 year old female and 
began to feel weakness in the left hand and developed mild-left hemiparesis due 
to infarction of right temporo-parieto-occipital lesion which was revealed by CT 
scan. Carotid angiograph showed irregularity at the right middle cerebral artery 
and 'string of beads' appearance of the right internal carotid artery. Gradually 
neurological manifestations improved, but a facial 'butterfly' rash, palmar 
erythema and polyarthritis were detected.(ABSTRACT TRUNCATED AT 250 WORDS)