[Clinical manifestations of infantile moyamoya disease]

The present study aimed to clarify clinical manifestations of infantile moyamoya disease by comparing them with those of schoolchild moyamoya disease.

This study included a total of 55 patients who were diagnosed as moyamoya disease on cerebral angiography. Their medical records were precisely evaluated. CT or MRI was performed to examine the location of cerebral infarction in all subjects. Cerebral blood flow was measured in 33 patients before surgery, using the 133-xenon inhalation method and single photon emission computed tomography (SPECT). Postoperative full-scale IQ (FSIQ) was also examined.

Of 55 patients, onset age was < 6 yr in 32 (infantile group) and > or = 6 yr in 23 (schoolchild group). Completed stroke was significantly more frequent in the infantile group than in schoolchild group (p = 0.0014). Incidence of cerebral infarct was also higher in the infantile group than in the schoolchild group (p = 0.0023). PCA stenosis/occlusion was observed in 14 of 32 infantile patients, but only in 4 of 23 schoolchild patients (p = 0.0474). Mean cerebral blood flow value was significantly lower than control value in the infantile group, but not in the schoolchild group. The effect of bypass surgery on cerebral ischemic attack was satisfactory in both groups. However, the postoperative FSIQ value was significantly lower in the infantile group than in the schoolchild group (p = 0.0452).

The present study showed that infantile moyamoya disease involves a higher risk for cerebral infarct and completed stroke due to its more advanced stage, and that earlier diagnosis and treatment should be performed to improve the mental prognosis of patients.