目的 研究肝豆状核变性患者胼胝体病变及其临床价值.方法 收集3例肝豆状核变性患者的临床及影像资料,参照2001年第八届莱比锡肝豆状核变性国际会议制定的诊断评分系统评分.利用磁共振成像(MRI)技术检查胼胝体压部病变.结果 3例患者均存在胼胝体压部病变,但均无胼胝体病变所致的失连接综合征表现.头颅MRI技术提示3例患者胼胝体病变主要为长T2异常信号、FLAIR像稍高信号,其中1例DWI呈高信号.出现胼胝体病变的3例肝豆状核变性患者均病情较重、脑部损伤弥漫(尾状核、壳核、苍白球、丘脑、中脑及桥脑).结论 胼胝体病变为肝豆状核变性患者少见的影像学表现,若在有典型基底节病变的同时还伴有胼胝体病变则高度支持肝豆状核变性的诊断.胼胝体病变也提示患者病情较重、脑部损伤弥漫,预后相对较差.
Aim To study the corpus callosum abnormalities and its clinical meaning in Wilson' s disease (WD).Methods Clinical and imaging data in three patients were collected according to the diagnosis scoring system of WD which was set up in Leipzid in 2001.Using magnetic resonance imaging (MRI),the splenium of the corpus callosum lesions were found in 3 patients.Results The splenium of the corpus callosum abnormality in WD is rare,but all the patients had no the manifestation of clinical dysconnexion syndrome.Cranial MRI showed the corpus callosum abnormality in 3 patients,high signal intensity in T2-weighted,slightly high signal intensity in fluid attenuated inversion recovery (FLAIR).Three patients had the same characteristics of more serious clinical symptom and diffuse brain abnormalities (caudate nuclei,putamen,thalamus,midbrain and pons).Conclusion The corpus callosum abnormalities are rare manifestation in WD.It is highly supported WD if patients appear the basal ganglia and corpus callosum lesions in magnetic resonance imaging.WD with abnormal corpus callosum also indicate that patients have bad condition,diffusive lesion in brain and possibly bad prognosis.