Moyamoya Syndrome with Recurrent Intraparenchymal Hemorrhage from Hemoglobin C Disease

Moyamoya syndrome is an acquired idiopathic non-atherosclerotic vasculopathy that involves the terminal internal carotid artery and its branches. It is associated with numerous medical conditions such as hematologic abnormalities, autoimmune diseases, brain radiation, and tumors. These medical diseases cause chronic endothelial damage to cerebral vasculature resulting in both friability and stenosis of vessels, presenting as cerebral hemorrhage or infarction. There is an established link between moyamoya syndrome and hematologic diseases such as sickle cell and beta thalassemia. A few case reports of rare hemoglobin opathies have caused moyamoya syndrome; however, there are no reports of moyamoya due to hemoglobin C disease. This case report presents a patient with known hemoglobin C disease who presented with her second intracranial hemorrhage, found to have moyamoya syndrome.