Moyamoya: Report of a Pediatric Case

Moyamoya (a japanese term, meaning ‘hazy things’) was first described by takeuchi in 1963.two forms of this disease have been distinguished: 1-primary moyamoya, or moyamoya disease,with a strong hereditary predisposition and girls are more frequently affected. 2-secondary moyamoya, or moyamoya syndrome, which is caused by a variety of underlyingdiseases.the japanese scientists have classified moyamoya into four types: hemorrhagic, epileptic,infarct, and transient ischemic attack.herein, we introduce an 8-years-old girl with the chief complaint of speech disorder. in herphysical examination, we detected expressive aphasia and right-sided central facial palsy.after a few days, right hemiplegia and cortical blindness appeared as well. gradually she wastotally unable to move and was transferred to the icu because of loss of consciousness.mri showed diffuse hyper signal lesions in the left temporoparietal and bilateral occipitalarea. mra showed narrowing of the internal carotid artery and abnormal collaterals (moyamoyavessels). after indirect bypass surgery (edas), she is now able to sit, walk, run andspeak.there are rare angiographically proven moyamoya cases. to our knowledge this was the firstedas in iran and a rare case of moyamoya with a dramatic response to operation.