肝豆状核变性的颅脑MRI 诊断与鉴别诊断

目的 回顾性分析肝豆状核变性的MRI诊断和主要鉴别诊断。方法 本组30例，男16例，女14例；临床表现；行走不稳，言语不清，共济失调，肌张力改变。结果 病变累及豆状核（30／30），尾状核（26／30）、丘脑（17／30），中脑（21／30）、桥脑（17／30）、丘脑外侧核（7／30）和脑白质（4／30）；脑萎缩以尾状核（14／30）和桥脑（11／30）为主，结论 基底节对称性异常信号伴有脑干病灶是肝豆状核变性的主要表现，鉴别诊断包括中毒性脑病和缺氧性脑病等。

Craniocerebral MRI Diagnosis and Differential Diagnosis on Hepatolenticular Degeneration

Objective MRI diagnosis and main differential diagnosis of hepatolenticular degeneration was analysed retrospectively. Methods 30 cases (16 male,14 female) of hepatolenticular degeneration were diagnosed.Their clinical symptom includes walking unstable,speech disorder,ataxia,dystonia. Results Nucleus lentiformis (30/30),caudate nucleus (26/30), thalamus (17/30),midbrain (21/30),pons (17/30),thalamus external nucleus (7/30),white matter of brain (4/30) were the usual involve areas.Brain atrophy in caudate nucleus (14/30) and pons (11/30) can be seen as wall. Conclusion Main manifestation of hepatolenticular degeneration were abnormal signal in bilateral basal ganglia and brain stem.Hepatolenticular degeneration should be differential diagnosed form toxic encephalopathy and anoxic encephalopathy. [