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目的探讨成人烟雾病(MMD)的临床和影像学特征。方法回顾性总结2006年1—12月在山东大学齐鲁医院经脑血管造影证实的16例成人MMD患者的临床及影像学特征。结果 16例患者平均年龄为(40.8±9.8)岁;15例(93.75%)以缺血性脑血管病为首发症状;1例(6.25%)以非典型血管病为首发症状;无脑出血患者。颅内有梗死灶或出血性梗死灶者12例(85.7%);双侧颈内动脉(ICA)末端、大脑中动脉(MCA)和(或)大脑前动脉(ACA)起始部狭窄或闭塞、伴有脑底部异常血管网及侧支循环形成的13例(92.9%);仅表现为脑动脉硬化者1例(7.1%)。双侧ICA末端及ACA、MCA起始部不同程度狭窄或闭塞者10例(62.5%);单侧血管病变者6例(37.5%);可见不同程度的侧支循环形成者15例(93.8%);烟雾状血管形成者13例(81.2%)。结论成人MMD患者的临床表现以缺血性症状为主,发病年龄高峰在40岁左右。影像学特征为双侧ICA狭窄或闭塞,伴有MCA或ACA的狭窄或闭塞,颅底有不同程度异常增生的血管网。对于青壮年反复出现脑梗死者,应考虑MMD的可能并及早行MRA或DSA检查。
Objective To investigate the clinical and imaging features of adult moyamoya disease (MMD). Methods The clinical and imaging features of 16 adult MMD patients confirmed by cerebral angiography at Qilu Hospital of Shandong University from January to December in 2006 were retrospectively reviewed. Results The average age of 16 patients was (40.8 ± 9.8) years old. Fifteen patients (93.75%) had ischemic cerebrovascular disease as the first symptom and one patient (6.25%) had atypical vascular disease as the first symptom. In patients without cerebral hemorrhage . Intracranial infarction or hemorrhagic infarction in 12 cases (85.7%); bilateral carotid artery (ICA) terminal, middle cerebral artery (MCA) and (or) anterior cerebral artery (ACA) initial stenosis or occlusion , 13 cases (92.9%) accompanied by abnormal blood vessels network at the base of the brain and formation of collateral circulation; only 1 case (7.1%) showed cerebral arteriosclerosis. There were 10 cases (62.5%) with different degrees of stenosis or occlusion at the beginning of bilateral ICA and ACA and MCA; 6 cases (37.5%) with unilateral blood vessel disease; 15 cases (93.8% ); Smoke-like angiogenesis in 13 cases (81.2%). Conclusion The clinical manifestations of adult MMD patients with ischemic symptoms, the peak age of onset in the 40-year-old. Imaging features of bilateral ICA stenosis or occlusion, with MCA or ACA stenosis or occlusion, skull base with varying degrees of abnormal proliferation of vascular network. For recurrent cerebral infarction in young adults should consider the possibility of MMD and early MRA or DSA examination.