Cerebrovascular arteriopathy in Progeroid Syndrome

초록

Progeroid syndrome also known as Hutchinson-Gilford syndrome is a very rare autosomal domindisorder that causes children to age faster, usually starting in their first one or two years. The patprogeria feature typically live to an age of early twenties. limited growth, alopecia and small face are all characteristics of progeria. Although patients show no neurodegeneration or cancer predishave early morbidity from cardiovascular and cerebrovascular disease. Here, we described the 5-yprogeria feature confirmed by genetic test, who have cerebrovascular arteriopathy without stroke The patient, aged five years with progeria feature was admitted to the Department of Pediatrics aconfirmed as Hutchinson-Gilford progeria syndrome by genetic test. He has typical progeria featustature, hair loss and small face but did not have stroke or cardiovascular symptoms. He was evaorgan status by multidisciplinary department team, including brain MR scans. MR images showedstenosis in bilateral cavernous segment of internal carotid artery with ivy signs, suggesting leptomcollaterals without encephalomalacia. Also, severe stenosis in bilateral vertebral arteries with collaenlarged anterior spinal artery was revealed. We recommened the weigh-adjusted clopidogrel anPatients with progeria usually develop atherosclerosis and die of cardiac or cerebrovascular diseaseven and early twenties years of age. Although the pathology of cerebrovascular disease is relatatherosclerosis, the efficacy and safety of statin and antithrombotics remain still unclear and the rbe needed in the future