Abstract
A pathological study on 10 autopsy cases of Moyamoya disease is reported. Two cases were children under 15 years old, and 8 cases adults. Six of 8 adult cases died of intracerebral hemorrhage. Bleeding point of 3 cases was suspected to be putamen, 2 cases thalamus and one claustrum. It was verified that hematoma ruptured into the lateral ventricles in all the cases.
Microsection of the affected main cerebral arteries at the base of the brain showed a marked thickening of the intima due to cellular or fibrous tissue, a tortuous appearance and reduplication of the internal elastic fibers and an atrophy of the media. No difference was found on pathological findings of arteries between children and adults. Some of the perforating arteries, which consisted basal moyamoya vessels, were dilated, while the other were occluded due to thickening of intima.
In 2 cases, main arteries in the whole body were examined. Similar changes to those of the cerebral arteries were observed in renal, coronal and aortic arteries. Therefore, it is possible that Moyamoya disease would be the manifestation of the general vasculitis at the brain.
