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日本无症状人群中的成人烟雾病

Adult moyamoya disease in the asymptomatic Japanese population.

作者信息

Ikeda Ken, Iwasaki Yasuo, Kashihara Hidetoshi, Hosozawa Ken-Ichi, Anan Kouzo, Tamura Masaki, Satoyoshi Eijiro, Ikeda Hidetoshi

机构信息

Department of Neurology, PL Tokyo Health Care Center, 16-1, Kamiyamacho, Shibuyaku, Tokyo 1500047, Japan.

出版信息

J Clin Neurosci. 2006 Apr;13(3):334-8. doi: 10.1016/j.jocn.2005.02.026. Epub 2006 Mar 15.

DOI:10.1016/j.jocn.2005.02.026
PMID:16540331
Abstract

Moyamoya disease (MMD) is a spontaneous occlusive disease of the Willis circle. This study evaluated the presence of the radiological hallmarks of adult MMD on a "brain check-up" (BC) using MRI and MR angiography, in Japan. BC was offered to 11,402 healthy subjects (7570 men and 3832 women) between January 1997 and November 2003. The mean age of all subjects was 53.2 years (SD 11.1); the mean age of men was 53.2 years (SD 10.9) and women 53.2 years (SD 11.5). MMD was diagnosed according to the criteria of the Research Committee on Spontaneous Occlusion of the Circle of Willis in Japan. Eight subjects (4 men and 4 women) were diagnosed as probable MMD without neurological deficits. Thus, the percentage of subjects with asymptomatic MMD on BC was 0.07% for the total group, 0.05% in men and 0.10% in women. The female to male ratio was 3.3:1. The mean age of the MMD patients was 54.0 years (SD 12.0); men 54.8 years (SD 12.8) and women 53.3 years (SD 13.9). Estimates of the prevalence of MMD in the Japanese population were calculated as 50.7 per 100,000 people; 28.9 in men and 94.3 in women. Five patients had a family history of definite MMD and seven had a family history of subarachnoid or intracerebral hemorrhage. MRI and angiography showed stenosis of the bilateral internal carotid arteries (ICA) in five patients and a unilateral ICA in three. Moyamoya vessels were confirmed on cerebral angiography. Our standard BC protocol is limited for the detection of moyamoya vessels in the initial stages of MMD. The present study suggests that the radiological features of MMD are asymptomatic in the early stages of the disease.

摘要

烟雾病(MMD)是一种Willis环的自发性闭塞性疾病。本研究在日本评估了使用MRI和磁共振血管造影在“脑部检查”(BC)中成人烟雾病放射学特征的存在情况。在1997年1月至2003年11月期间,对11402名健康受试者(7570名男性和3832名女性)进行了脑部检查。所有受试者的平均年龄为53.2岁(标准差11.1);男性平均年龄为53.2岁(标准差10.9),女性为53.2岁(标准差11.5)。烟雾病根据日本Willis环自发性闭塞研究委员会的标准进行诊断。8名受试者(4名男性和4名女性)被诊断为可能患有烟雾病但无神经功能缺损。因此,脑部检查中无症状烟雾病受试者的比例在总人群中为0.07%,男性为0.05%,女性为0.10%。女性与男性的比例为3.3:1。烟雾病患者的平均年龄为54.0岁(标准差12.0);男性为54.8岁(标准差12.8),女性为53.3岁(标准差13.9)。日本人群中烟雾病患病率的估计值计算为每10万人中有50.7例;男性为28.9例,女性为94.3例。5名患者有明确的烟雾病家族史,7名患者有蛛网膜下腔或脑出血家族史。MRI和血管造影显示5例患者双侧颈内动脉(ICA)狭窄,3例患者单侧ICA狭窄。脑血管造影证实有烟雾血管。我们的标准脑部检查方案在烟雾病早期阶段检测烟雾血管方面存在局限性。本研究表明,烟雾病的放射学特征在疾病早期是无症状的。

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