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 dis-ease, with a strong hereditary predisposition and girls are more frequently affected. 2-Secondary moyamoya, or moyamoya syndrome, which is caused by a variety of underlying dis-eases. 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 her physical 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 was totally 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 occipital area. MRA showed narrowing of the internal carotid artery and abnormal collaterals (moyamoya vessels). After indirect bypass surgery (EDAS), she is now able to sit, walk, run and speak. There are rare angiographically proven moyamoya cases. To our knowledge this was the first EDAS in Iran and a rare case of moyamoya with a dramatic response to operation.