Postpartum Ischemic Stroke in Moyamoya Disease Associated with Protein Z Deficiency-A Case Report.

The authors describe herein the first adult case with moyamoya disease associated with protein Z deficiency. A 41-year-old woman was admitted to our hospital due to sudden onset of dysarthria and left extremity weakness 6 days after the delivery of her first baby. Previously, she repeated early fetal losses and was diagnosed with protein Z deficiency. Laboratory examination showed that the plasma concentration of protein Z was .73 µg/ml, being lower than the control. Radiological examination demonstrated typical findings of moyamoya disease with advanced stage on both sides. She successfully underwent surgical revascularization on both sides and was free from any cerebrovascular events during a follow-up period of 2 years. In addition to hemodynamic compromise, protein Z deficiency and hypercoagulation state after delivery might cause ischemic stroke in this case.