豫西南地区烟雾病的临床特征分析

Clinical Features of Moyamoya Disease in Southwest Henan Province

目的分析豫西南地区烟雾病(MMD)的临床特征。方法选取2017—2021年南阳市中心医院收治的418例MMD患者为研究对象,收集其临床资料,包括性别、发病年龄、民族、地区、临床表现(包括临床表现分型和首发症状)及影像学表现。结果418例患者中男女比例为1∶1.06;儿童14例(3.3%),成人404例(96.7%);发病年龄有两个高峰,分别为5~9岁、45~54岁;汉族417例,满族1例;唐河县130例,南阳市区69例,社旗县46例,方城县44例,新野县34例,邓州市33例,其他地区62例;缺血型MMD 224例(53.6%),出血型MMD 132例(31.6%),单纯头痛型MMD 6例(1.4%),癫痫型MMD 5例(1.2%),无症状型MMD 5例(1.2%),其他分型MMD 46例(11.0%)。缺血型MMD患者首发症状为头晕、肢体无力最常见,分别占40.6%、34.4%;出血型MMD患者首发症状为意识障碍、头痛最常见,分别占42.4%、37.9%。缺血性MMD患者首发症状为头痛、意识障碍者所占比例低于出血型MMD患者,头晕、肢体无力、言语障碍、感觉障碍者所占比例高于出血型MMD患者(P<0.05)。418例患者影像学表现为双侧血管烟雾化367例(87.8%),单侧血管烟雾化51例(12.2%);同时存在大脑后动脉狭窄/闭塞者80例(19.1%)。结论豫西南地区女性MMD患者稍多于男性,发病年龄高峰为5~9岁和45~54岁,成人明显多于儿童;地区分布呈区域聚集性,唐河县最多;缺血型MMD患者的主要首发症状为头晕、肢体无力,出血型MMD患者的主要首发症状为意识障碍、头痛;MMD不仅累及前循环,也可累及后循环。

Objective To analyze the clinical features of moyamoya disease(MMD)in southwest Henan Province.Methods A total of 418 MMD patients admitted to Nanyang Central Hospital from 2017 to 2021 were selected as the research objects.Their clinical data,including gender,age of onset,nationality,region,clinical manifestation(including clinical manifestation classification and initial symptoms)and imaging manifestation were collected.Results The male to female ratio of 418 patients was 1∶1.06.There were 14 children(3.3%)and 404 adults(96.7%).There were two peaks in the age of onset,which were 5-9 years old and 45-54 years old.There were 417 cases of Han nationality and 1 case of Manchu nationality.There were 130 cases in Tanghe County,69 cases in Nanyang City,46 cases in Sheqi County,44 cases in Fangcheng County,34 cases in Xinye County,33 cases in Dengzhou City,and 62 cases in other regions.There were 224 cases(53.6%)of ischemic MMD,132 cases(31.6%)of hemorrhagic MMD,6 cases(1.4%)of simple headache type of MMD,5 cases(1.2%)of epileptic MMD,5 cases(1.2%)of asymptomatic MMD,and 46 cases(11.0%)of other type of MMD.The most common initial symptoms of ischemic MMD patients were dizziness and limb weakness,accounting for 40.6%and 34.4%,respectively.The most common initial symptoms of hemorrhagic MMD patients were disturbance of consciousness and headache,accounting for 42.4%and 37.9%,respectively. The proportion of ischemic MMD patients with the initial symptoms of headache and consciousness disturbance was lower than that of patients with hemorrhagic MMD, and the proportion of patients with dizziness, limb weakness, speech disorder and sensory disturbance was higher than that of patients with hemorrhagic MMD (P < 0.05) . Among the 418 patients, 367 cases (87.8%) had bilateral vascular image of smoke and 51 cases (12.2%) had unilateral vascular image of smoke;80 patients (19.1%) had posterior cerebral artery stenosis/occlusion. Conclusion Female MMD patients are slightly more than male MMD patients in southwest Henan Province, and the peak age of onset was 5-9 years old and 45-54 years old, and adults are significantly more than children. The regional distribution showed regional aggregation, Tanghe County is the most;the main initial symptoms of ischemic MMD are dizziness and limb weakness, while the main initial symptoms of hemorrhagic MMD are disturbance of consciousness and headache;MMD involves not only the anterior circulation, but also the posterior circulation.