抄録
A case of left occipital leptomeningeal angiomatosis was reported.
The patient was a 12-year-old boy who had episodes of severe vascular type headache accompanied by transient right homonymous hemianopsia. CT scan showed localized superficial high density area in the left occipital pole. Remarkable enhancement of the lower and inner surface of the left occipital lobe was demonstrated. Angiography showed poor filling of the distal portion of the left posterior cerebral artery. Skull tomograms showed linear calcifications in the left occipital region. Brain scan showed increased RI uptake in the left occipital region. During operation, the surface of the left occipital lobe was covered by excesive, fine, vascular networks which extended over the arachnoid membrane. The abnormal vessels were cauterized by a CO2 laser as throughly as possible. The occipital pole, felt gritty. Histologically, the abnormal vessels had spread into the subarachnoid space and were predominantly veins with thin and enlarged walls. The abnormal vessels followed the leptomeninges in the sulci of the cerebral cortex. Underneath the abnormal vessels, in the external layers of the cerebral cortex, calcium deposits were scattered and gliosis and degeneration of the ganglion cells were observed. The lesion was comparable with leptomeningeal angiomatosis. Though the pathological findings of the specimen, CT findings, and brain scan findings were extremely similar to those of Sturge-Weber disease, in this case, the typical clinical and roentgenographic findings of Sturge-Weber disease were all absent.
