[A case of progeria syndrome with cerebral infarction]

Cerebrovascular involvement is rare in progeria syndrome (Hutchinson-Gilford syndrome). A patient with progeria syndrome, who developed cerebral infarction, was reported. At 7 years of age, she suffered from right hemiplegia and transient ischemic attacks. X-ray CT showed multiple low density lesions in left frontal and parieto-occipital areas, which were enhanced with a contrast medium. Cerebral angiography demonstrated complete occlusion of left carotid artery and narrowing of vertebral artery. Brain imaging using 123I-N-isopropyl-p-iodoamphetamine (123I-IMP SPECT), which expressed regional cerebral blood flow, showed extensive perfusion defect over the left cerebral hemisphere in early scans, and redistribution phenomena in late scans. The symptoms improved gradually, which correlated well with 123I IMP-SPECT findings. This method will be useful to determine the prognosis as well as to understand changeable hemodynamic pathophysiology. A slowing of back ground on EEG also correlated well with clinical symptoms. It was necessary to examine the possibility of cerebrovascular involvement in progeria.