成人肝豆状核变性的颅脑MRI特征分析

The characteristics of MRI brain images in adult patients with hepatolenticular degeneration

目的分析成年患者肝豆状核变性的颅脑MRI表现。方法回顾性分析11例成年肝豆状核变性患者的颅脑MRI图像,所有患者均行T1WI、T2WI、FLAIR序列检查。结果 4例患者可见中脑"熊猫脸征",8例患者豆状核MRI表现轻微,仅表现为T2低信号,2例患者豆状核T2信号升高,并且信号混杂。11例患者均可见脑干T2信号升高,伴脑干萎缩。10例可见不同程度脑萎缩。2例患者额叶皮层缺血伴周围胶质增生。结论成年患者肝豆状核变性的颅脑MRI表现具有多样性,相较于儿童,成年患者小脑和中脑萎缩、脑干T2信号增高,豆状核T2低信号征象更多见,且具有重要的诊断意义。

Objective To analyze the characteristics of MRI brain images in patients with hepatolenticular degeneration（Wilson disease）. Methods The clinical data of MRI brain images of 11 patients with hepatolenticular degeneration were retrospectively analyzed,and all the patients underwent examination of T1 WI,T2 WI,FLAIR. Results Four patients presented midbrain＂face of the giant panda sign＂,and 8 cases presented minimum signal change of lenticular nuclei,just T2 hypointensity. Two cases presented mixed T2 hyperintensity of lenticular nuclei,and 11 patients showed T2 hyperintensity of brainstem and brainstem atrophy. Moreover 10 cases showed brain atrophy at different extents,and 2 cases showed lobus frontalis cortex ischemia complicated by surrounding gliosis. Conclusion The brain MRI characteristics in adult patients with Wilson disease are diversity. As compared with children,the cerebrum and brainstem atrophy,T2 hyperintensity of brainstem and T2 hypointensity of lenticular are more common in adult patients,which may have important diagnostic significance.