4例肝豆状核变性头颅核磁共振成像的非典型表现  

Four case reports of atypical cranial magnetic resonance imaging in Wilson's disease

作　　者：陈松林[1] 梁颖茵[1] 周香雪[1] 孙海兴[2] 李洵桦[1] 

机构地区：[1]中山大学附属第一医院神经内科,广东510080 [2]中山大学附属第一医院影像科,广东510080

出　　处：《世界临床药物》2010年第5期291-294,共4页World Clinical Drug

基　　金：广东省医学科研基金(No.A2006177);广东省自然科学基金(No.06300815)

摘　　要：目的评价4例肝豆状核变性(WD)头颅核磁共振成像(MRI)的非典型影像学表现,扩展对WD头颅MRI表现的认识。方法分析4例WD患者头颅MRI图像,归纳其MRI T1WI、T2WI和FLAIR(水抑制)成像特点,并记录其相应的临床表现。结果 4例患者头颅MRI表现为分布在额叶内异常信号呈片状或斑点状,或有囊腔形成,随访发现囊腔为可逆性改变;而基底节区异常信号相对较少。所有异常病灶在头颅MRI上呈低T1WI,高T2WI信号,FLAIR呈高信号,且均有脑萎缩改变。4例患者除有典型的锥体外系症状外,均出现智能障碍,3例合并癫痫发作。结论 WD患者MRI的非典型表现在基底节区以外,可以额叶和白质受累为主,并与某些临床表现有一定相关性。Objective To evaluate four case reports of atypical cranial magnetic resonance imaging （MRI） in Wilson＇s disease （WD）. Methods The cranial MRI characteristics ofT1WI, T2WI and FLAIR of four WD patients were described, and their clinical symptoms were reported. Results Cranial MRI features included atrophic changes and focal lesions. Most lesions were located in the frontal lobe instead of the basal ganglia. Lesions presented as flaky, macular or cyst shape, and cyst changes were reversible. Abnormal focus showed low-signa-intensity lesions on Tl-weighted, high-signal-intensity lesions on T2-weighted and high signal on FLAIR imaging. All patients showed intelligence decline and 3 patients complicated with epilepsy besides the typical extra pyramidal disturbances. Conclusion Frontal lobe rather than basal ganglia abnormal signals may be considered to be the atypical cranial MR/features in WD and to correlate with some clinical manifestations.

关 键 词：肝豆状核变性 WILSON病 核磁共振成像 

分 类 号：R445.2[医药卫生—影像医学与核医学] R742.4[医药卫生—诊断学]