成年人肝豆状核变性的颅脑MRI表现

目的分析21例成年人肝豆状核变性(HLD)的颅脑MR表现,以提高对HLD成年期的认识。方法 21例HLD患者中,所有病例均行MRI检查,其中3例作MRI增强检查。结果颅脑MRI检查21例中,20例有异常发现。累及豆状核(壳核及苍白球)20例,丘脑15例,中脑10例,额顶叶10例,桥脑7例,尾状核3例,胼胝体压部2例,小脑齿状核1例。20例合并脑萎缩;3例MRI增强检查中,2例未见异常强化,1例额叶病灶呈轻度脑回样强化。本组21例中,2例额叶病灶及1例小脑齿状核病灶稍不对称,其余病灶均呈两侧对称性分布。结论成年人HLD颅脑MRI表现具有一定特征性,即对称性的双侧豆状核、丘脑异常信号,可同时累及额顶叶白质,多伴不同程度的脑萎缩。

MRI Features of Brain with Hepatolenticular Degeneration in Adults

Objective To analyze the MR1 features in the brain of adult patients with HLD. Methods 21 patients with HLD were performed plain MRI scanning, and 3 patients received enhanced MRI scanning. Results Among the 2t patients, 2（} patients showed abnormally on MRI scans. There are 19 patients have lesions in the nucleus lentiformis （putamen and globus pallidum）, 15 patients in the thalami, 10 patients in the mesencephalon, 10 patients in the lobus frontalis and lobus parietalis, 10 patients in the pons, 3 patients in the caudate nuclei, 2 patients in the splenium corporis occipitalis, 1 patient in the dentate nucleus. 20 patients accompanied with brain atrophy. 2 of the patients who received enhancement scanning showed no abnormal enhancement while 1 patient showed gyria-like enhancement of the lobus frontalis. The lesions of the lobus frontails in 2 patients and the lesion of the dentate nucleus in 1 patient were unsymmetrical, while the distribution of the other lesions were bilateral and symmetrical in the other patients. Conclusion Brain MI〈I features of adults with HLD were that the symmetrical abnormal signal in the bilateral basal ganglia, thalami, and white matter of the lobus frontalis and lobus parietalis and most of the patients had brain atrophy to some extent.