以皮质表面铁沉积为影像特征的脑淀粉样血管病临床特点分析  

Clinical features of cerebral amyloid angiopathy characterized by cortical superficial siderosis

作　　者：李伟[1] 焦晟[2] 徐蕾[1] 陈玉辉[1] 王音[1] 龚涛[1] Li Wei;Jiao Sheng;Xu Lei;Chen Yuhui;Wang Yin;Gong Tao(Neurology Department,Beijing Hospital,National Center of Gerontology,Beijing 100730,China;Radiology Department,Beijing Hospital,National Center of Gerontology,Beijing 100730,China)

机构地区：[1]北京医院,国家老年医学中心,神经内科,北京100730 [2]北京医院,国家老年医学中心,放射科,北京100730

出　　处：《中华医学杂志》2019年第18期1397-1400,共4页National Medical Journal of China

摘　　要：目的研究以皮质表面铁沉积为特征的脑淀粉样血管病的临床和影像特点,提高临床医生对本病的认识。方法回顾性分析2013年6月至2016年8月北京医院收治的以皮质表面铁沉积为特征的16例脑淀粉样血管病患者,分析流行病学资料、临床特点、头颅磁共振成像(MRI)及脑电图检查结果等信息。结果16例患者男女比例为1.67∶1,发病年龄73(69~79)岁,最常见的临床症状为短暂性局灶性神经系统症状发作(TFNE,12/16)。头颅MRI表现为局限型(9/16)和弥漫型皮质表面铁沉积(7/16);可伴有不同程度的脑微出血。脑电图检查多数结果正常(6/9),少数表现为局灶性慢波(3/9)。随访17(17±11)个月,5例患者出现反复TFNE发作,其中1例发生脑出血。结论以皮质表面铁沉积为特征的脑淀粉样血管病多见于老年人,短暂性局灶性神经系统症状发作为最常见的临床表现,头颅MRI是最重要的诊断方法,应尽量避免抗栓治疗。Objective To study the clinical and imaging characteristics of cerebral amyloid angiopathy characterized by cortical superficial siderosis and improve clinicians′ understanding of the disease.Methods Retrospective analysis was performed on 16 patients with cerebral amyloid angiopathy characterized by cortical superficial siderosis from June 2013 to August 2016 in Beijing Hospital,and the information including epidemiological data,clinical features,cranial MRI and electroencephalogram (EEG) results were analyzed.Results The ratio of male to female in 16 patients was 1.67∶1,and the average age of onset was 73 (69-79) years.The most common clinical symptoms were transient focal neurological episodes (TFNEs)(12/16).Cranial MRI showed localized (9/16) and diffuse type cortical superficial siderosis (7/16);few of them were associated with different degrees of cerebral microbleeds.Most of the EEG findings were normal (6/9) and a few showed focal slow waves (3/9).During a mean follow-up of 17 (17±11) months,5 patients developed repeated TFNE,of which 1 had cerebral hemorrhage.Conclusions Cerebral amyloid angiopathy characterized by cortical superficial siderosis occurs predominantly in the elderly.TFNE is the most common clinical manifestation.Cranial MRI is the most important diagnostic method,and antithrombotic therapy should be avoided as much as possible.

关 键 词：皮质表面铁沉积 脑淀粉样血管病 短暂性局灶性神经系统症状发作 

分 类 号：R742[医药卫生—神经病学与精神病学]