[en] In 4 Sturge-Weber-syndrome child patients with calcified lesions mainly in the occipital lobe, regional cerebral blood flow (rCBF) was determined with stable Xe-CT in the resting state and after iv injection of acetazolamide (AA) and megimide (M), with the purpose of examining factors affecting deterioration of neurologic manifestations and the influence of lobectomy. In the resting state, rCBF of the temporal and occipital areas was significantly lower on the affected than unaffected sides. The side-to-side asymmetry of rCBF in the affected and unaffected sides decreased from the frontal to temporal and occipital areas. It was indistinct after AA injection because cerebral vasoreactivity became higher on the affected side. A low rCBF in association with a high cerebral vasoreactivity on the lesion side suggested that the low rCBF matched the low cerebral metabolism of the brain area affected by leptomeningeal angiomatosis. However, cerebral vasoreactivity to AA depended on clinical presentations. Two patients presented with progressive mental retardation. The other two patients were in clinically stable condition. Cerebral vasoreactivity to AA in the former two cases was poorer on both the affected and unaffected sides than that in the latter two cases. M administration was associated with a significantly decreased rCBF in the area of leptomeningeal angiomatosis and cerebral calcification and with a significantly increased rCBF of the 'pericalcified' area. In one patient undergoing extended occipital lobectomy on the affected side, no decrease in rCBF was noted in the resting state in either affected or unaffected side, and cerebral vasoreactivity to AM on the unaffected side was apparently increased. Both circulatory disturbance and seizure appear to play a role in clinical presentations, as well as their deterioration in Sturge-Weber-Syndrome patients. (N.K.)