成年人烟雾病进展的临床分析

Progression of adult moyamoya disease

目的探讨成年人烟雾病进展的临床特征。方法总结我院42例成年人烟雾病患者，其中40例患者纳入研究，33例为双侧病变，7例为单侧病变，所有患者平均随访70．2个月．1-2年采用磁共振成像、磁共振血管造影进行随访检查，发现可疑疾病进展时则行数字减影血管造影检查。根据疾病进展情况把患者分成进展组和稳定组。结果在随访期间，40例患者中8例出现病情进展，单侧及双侧病变均可出现疾病进展，前、后循环均可累及，8例患者中7例出现与病情进展相关的再出血或缺血。单因素检验表明性别、初次发病年龄、发病表现（缺血或出血）、病变血管类型（单侧或双侧）、手术（脑颞肌贴覆术）治疗在疾病进展和稳定2组中均无统计学意义（P〉0．05）。结论成年人烟雾病患者病情进展的发生率高于以往认识，进行长期随访，及早发现进展、预防再次中风、提高预后是很必要的。

Objective To observe the clinical progression of adult moyamoya disease（MMD）. Methods Forty MMD patients were enrolled. Among them, 33 patients were confirmed with typical “bilateral” moyamoya disease. The other 7 patients were suspected of “unilateral” moyamoya disease. All cases were followed up with a mean period of 70.2 months. Magnetic Resonance Imaging （MRI） and Magnetic Resonance Angiography （MRA） were repeated every 1 to 2 years, and Digital subtraction angiography （DSA） was performed when MRI and MRA showed possible disease progression. Results Disease progression occurred in 8 of 40 patients （20%） during the foBow-uPS. Occlusive arterial lesions progressed in both anterior and posterior brain vessels. In both bilateral and unilateral groups. 7 of 8 patients developed ischemic or hemorrhagic events. Between progression group and stable group, no significant difference was shown in terms of gender, age, onset symptoms （Ischemia or Bleeding） ,type of disease （bilateral or single-sided） and bypass surgery. Conclusion The incidence of progression of adult moyamoya disease is much higher than previously recognized, Neurological and radiological follow-ups would be essential in adult movamova patients for stroke prevention and better outcomes.