Abstract
In 21 children with Moyamoya disease, intellectual examinations and measurements of regional cerebral blood flow (rCBF) were performed to evaluate whether a chronic hypoperfusion in developing brain results in a progressive intellectual deterioration or not. These children with the disease included 9 males and 12 females, and the clinical types at the onset included transient ischemic attack (TIA) type (17 cases), infarction type (2 cases), infarction+epilepsy type (1 case) and epilepsy type (1 case). These 21 children were divided into two groups according to the age at onset: a younger group with ages under 6 and an elder group with ages 6 and over. The mean IQ of the younger group was 87 and significantly lower than that of the elder group (123). The patients who suffered from this disease for a longer time and who belonged to infarction type or epilepsy type tended to have lower IQ. There were patients who had high IQ in spite of the low level of rCBF, and vice versa. Thus, there was no definite relationship between the level of rCBF and IQ in this study In patients who received intracranial-extracranial bypass surgery, though rCBF increased by approximately 10%, IQ was unchanged or improved only slightly. In this study, the poor mental prognosis was correlated with early onset in Moyamoya disease. Therefore, early diagnosis and early treatments are of significant importance in this disease.
