Symptoms and their pattern of progression in childhood moyamoya disease.

In the series of 81 childhood moyamoya patients, the common pattern of clinical progression seen in 77 patients was revealed to be no more than four. The characteristics of each type of clinical progression were presented for a better understanding of the disease and for an initiation of early treatment of this rather rare and insidiously progressive disease. Type I (21 cases) and type II (15 cases) are cases without infarction, even in those types there are cases with poor IQ. Type III (17 cases) and type IV (24 cases) are cases with infarction and an early operation is mandatory. Types I and II are also good candidates for early operation, for IQ deterioration insidiously progress in these types and there is no way of knowing them from type III before infarctions occur. We now have effective surgical measures to improve the ischemic conditions of this disease.