烟雾病图文课件_3.pptVIP

Moyamoya Disease Content Definition Etiology Pathophysiology Clinical Manifestation Diagnosis Treatment Prognosis Definition   Moyamoya Disease is a disease in which certain arteries in the brain are constricted. Blood flow is blocked by the constriction, and also by blood clots (thrombosis). A collateral circulation develops around the blocked vessels to compensate for the blockage, but the collateral vessels are small, weak, and prone tohemorrhage, aneurysm and thrombosis. On conventional X-ray angiography, these collateral vessels have the appearance of a puff of smoke (described as もやもや (moyamoya) in Japanese) Etiology About 10% of cases of moyamoya disease are familial, and some cases result from specific genetic mutations.  In Japan the overall incidence is higher (0.35 per 100,000). Moyamoya disease can be either congenital or acquired. Patients with Down syndrome, sickle cell anemia, neurofibromatosis type 1, congenital heart disease,fibromuscular dysplasia, activated protein C resistance, or head trauma can develop moyamoya malformations. It is more common in women than in men, although about a third of those affected are male. Pathophysiology The disease moyamoya makes the blood leak out of the arteries, causing pressure to the brain and subsequent headaches.  The pathogenesis of moyamoya disease is unknown, although the gene ring finger protein 213 (RNF213) has been implicated. Once it begins, the vascular occlusion tends to continue despite any known medical management. In some people this leads to transient ischemic attacks or repeated strokes with severe functional impairment or even death. In others, the blockage may not cause any symptoms. The disease causes constrictions primarily in the internal carotid artery, and often extends to the middle and anterior cerebral arteries, branches of the internal carotid artery inside the skull. When the internal carotid artery becomes completely blocked, the fine coll