肝豆状核变性的脑部MRI表现（附13例报告）  

Brain MRI Manifestations in Hepatolenticular Degeneration(A Report of 13 Cases)

作　　者：程敬亮[1] 李树新[1] 任翠萍[1] 王秀玉[1] 李荫太[1] 汪浒[1] 郭攻 

机构地区：[1]河南医科大学第一附属医院放射科,郑州铁路中心医院磁共振室

出　　处：《中华放射学杂志》1995年第8期511-514,共4页Chinese Journal of Radiology

摘　　要：分析１３例肝豆状核变性（ＨＬＤ）脑部ＭＲＩ表现特点以及脑部病变分布和病程间的关系。病程０．１７至９．０年，分为１年内组（７例）和１年后组（６例）。１３例ＨＬＤ壳核全部受累，其次为尾状核（８５％）、丘脑（７７％）、红核（６９％）、苍白球（３１％）、导水管周围灰质（２３％），桥脑（２３％）和黑质（１５％）。其分布特点为双侧对称性，ＭＲＩ多呈长Ｔ＿１和长＿２信号。壳核病变表现特殊，其外缘呈更长Ｔ＿１和更长Ｔ＿２信号，勾画出壳核外缘轮廓。结果显示，ＨＬＤ脑部病变分布与病程间无相关性。ＭＲＩ不仅是ＨＬＤ脑部病变检出的有效方法，并可随访其疗效。Ｔ＿２－ＷＩ是检出ＨＬＤ脑部病变的最佳序列。壳核病变的特殊表现有助于ＨＬＤ诊断。The brain MRI features of 13 patients with hepatolenticular degeneration (HLD)proven clinically and biochemically were presented, possible correlation between brain lesion sites and duration of disease was analysed. Duration of the disease varied from 0.17 year to 9.0 years in 13 HLD. They were divided into two groups : within 1 year (7 cases) and over 1 year ( 6 cases) . The putamen was affected in all cases. Other affected sites and their incidence were caudate nucleus(85% ), thalamus (77%) , red nucleus (69%), globus pallidum (31%) , periaqueductal grey matter(23%), pons (23%) and substantia nigra (15% ) respectively. The distribution of lesions were char-acteristically bilateral and symmetric. In the majority of cases, the lesions exhibited low signal on T_1 weighted image and high signal on T_2-weighted image even more so at the peripheral part of puta-men, so that the lateral border of putamen was delineated. The results demonstrated that MRI was an efficient method for studying in brain lesions in HLD and evaluating therapeutic efficacy. There was no positive correlation between brain lesion sites and duration of disease in our cases, The char-acteristic MRI appearance of putamen would be beneficial to the diagnosis of HLD.

关 键 词：肝豆状核变性 NMR 成像 脑 

分 类 号：R742.404[医药卫生—神经病学与精神病学]