肝豆状核变性12例颅脑MRI诊断

Craniocerebral MRI Diagnosis on Hepatolenticular Degeneration

目的:分析肝豆状核变性(HLD)的颅脑MRI征象,进一步提高对HLD病的认识及诊断的正确率。方法:全部病历均行MRI扫描,回顾性分析其MRI征象及临床表现。结果:6例豆状核头部及壳核出现对称性条带状或新月行长T1、长T2、FLAIR高信号,形如“八字”或“展翅蝴蝶”样;4例丘脑对称性卵圆形长T1、长T2、FLAIR高信号;2例尾状核头部、壳核、丘脑、小脑齿状核及脑干对称性长T1、长T2、FLAIR高信号。全部病例均有不同程度的脑沟裂增宽等脑萎缩表现。结论:HLD颅脑MRI最多见征象是脑萎缩与基底节异常信号,特别是双侧豆状核区对称性长T1、长T2、FLAIR高信号最具有特征性。

Objective To improve the MRI diagnostic accuracy and the understanding of hepatolenticular degeneration （HLD） by analyzing the magnetic rescnance imaging （MRI） features. Methods 12 cases of HLD proved clinically went through the Craniocerebral MRI examination including TIWI,T2WI and FLAIR sequences. Both MRI and clinicaldata were evaluated retrospectively. Results Abnormal signals on Craniocerebral MRI included long T1 signal, long T2 signal and hyperino tensity on fluid attentedinversionrecovery（FLAIR）. Six cases showed the symmetrical signal abnormal in bilateral globus pallidum and putamen, four cases in bilateral thalamus, two cases in bilateral caudate head, globus pallidum, dentate nucleus of cerebella,thalamus and brain stem. At the same time,various degrees of encephalatrophy were seen in all cases. Conclusion The familiar craniocerebral MRI features of HLD include encephalatrophy and abnormal signals in basal segment region,especially the symmetrical long T1 signal, long T2 signal and hyperintensity on FLAIR in bilateral lentiform nucleus.