Ueki K, Meyer F B, Mellinger J F
Department of Neurologic Surgery, Mayo Clinic Rochester, Minnesota 55905.
Mayo Clin Proc. 1994 Aug;69(8):749-57. doi: 10.1016/s0025-6196(12)61094-5.
To discuss the clinical features of moyamoya disease, the studies that aid in diagnosing this disorder, and the reported outcomes of surgical treatment.
We review the manifestations of moyamoya disease in children and adults and the recent reports of the various surgical procedures.
Moyamoya disease is a chronic cerebrovascular disorder in which stenosis of the major arteries of the circle of Willis at the base of the skull progresses to occlusion. The diagnosis is based on the angiographic findings of the "puff of smoke" appearance of the abnormal capillary vessels at the base of the skull. Three surgical procedures are used to manage this disease: anastomosis of the superficial temporal artery to the middle cerebral artery, encephalomyosynangiosis, and encephaloduro-arteriosynangiosis.
In children with this disease, cerebral ischemic events, including strokes, occur. In adults, the fragile abnormal vessels can rupture and cause intracerebral hemorrhage. The mortality rate for adults is higher than that for children. Most published reports support the efficacy of surgical treatment in children but not in adults.
The natural history of moyamoya disease is poor; neurologic deterioration due to strokes and hemorrhage is progressive. Seizures and intellectual deterioration can occur.
探讨烟雾病的临床特征、有助于诊断该疾病的研究以及手术治疗的报道结果。
我们回顾了儿童和成人烟雾病的表现以及各种手术方法的近期报道。
烟雾病是一种慢性脑血管疾病，颅底 Willis 环主要动脉的狭窄会逐渐发展为闭塞。诊断基于颅底异常毛细血管呈现“烟雾状”外观的血管造影结果。有三种手术方法用于治疗这种疾病：颞浅动脉与大脑中动脉吻合术、脑-肌-血管融合术和脑-硬膜-动脉血管融合术。
患有这种疾病的儿童会发生包括中风在内的脑缺血事件。在成人中，脆弱的异常血管可能破裂并导致脑出血。成人的死亡率高于儿童。大多数已发表的报告支持手术治疗对儿童有效，但对成人无效。
烟雾病的自然病程较差；中风和出血导致的神经功能恶化是渐进性的。可能会发生癫痫发作和智力衰退。
