Disease Information for MoyaMoya disease: Definition

  • A chronic cerebral vasculopathy primarily occurring in childhood and characterized by slowly progressive carotid artery narrowing and occlusion at the base of the brain. An extensive network of anastomoses forms between branches of the carotid arteries, resulting in a characteristic angiographic appearance; The condition may be idiopathic (classic moyamoya disease) or occur in association with ANEMIA, SICKLE CELL; DOWN SYNDROME; CRANIOCEREBRAL TRAUMA; RADIOTHERAPY induced arterial injury; NEUROFIBROMATOSIS; and other diseases; Clinical manifestations include hemiparesis, headache, seizures, and delayed mental development; In older individuals, this disease may present as SUBARACHNOID HEMORRHAGE; (From Adams et al, Principles of Neurology, 6th ed, p831; Clin Neurol Neurosurg 1997 Oct;99 Suppl 2:S36-8)---------------------------------------.

    Moyamoya Syndrome; Moya-moya Disease; Moyamoya syndrome is a progressive disorder that affects the blood vessels in the brain (cerebrovascular); It is characterized by the narrowing (stenosis) and/or closing (occlusion) of the carotid artery, the major artery that delivers blood to the brain; Inadequate blood supply leads to reduced oxygen to the brain, and it is this oxygen deprivation that causes the signs of Moyamoya; Those signs most typically include paralysis of the feet, legs or the upper extremities; Headaches, various vision problems, mental retardation, and psychiatric problems may also occur;

    Approximately 10% of cases of Moyamoya syndrome are due to a genetic cause and are termed primary Moyamoya syndrome; . Secondary Moyamoya syndrome refers to cases in which the syndrome is a consequence or result of another underlying disorder; In secondary Moyamoya syndrome, when it is not a result of a genetic cause, it is important for the physician to determine the root underlying cause; [NORD 2005]----------------------------------------------