Ikeda K, Sakata C, Nemoto H, Yokoi F, Sunohara N, Iio M
Department of Neurology, National Center of Neurology and Psychiatry.
Rinsho Shinkeigaku. 1991 Feb;31(2):147-53.
Follow-up magnetic resonance imaging (MRI) and computed tomography (CT) examinations were performed on five patients with Wilson's disease at intervals from 6 to 29 months. We studied the clinical correlation with MRI and CT, and whether the examination of MRI and CT could be useful for evaluation of the therapeutic effect. Positron emission tomography (PET) was also carried out on 4 cases except for an asymptomatic case (patient 2, sister of patient 1). Close relationship has been observed by MRI between dystonia and the lesion of the lenticular nuclei, abnormality of smooth pursuit eye movements and the brain stem lesion, and severe dysarthria/dysphagia and the lesion of the caudate and lenticular nuclei, respectively. In patient 4, repeated MRI of an interval of 18 months demonstrated decrease of the abnormal high signal in the lateral part of the putamen on T2-weighted image in accordance with marked improvement of clinical manifestations. In patient 3, who had severe dystonia of the extremities and trunk, T2-weighted image showed high signals in the lenticular nuclei. Marked decrease of the high signal in the lenticular nuclei was observed by MRI in this patient after 29 months, when her neurological manifestations were markedly improved. Patient 5 with severe cerebellar signs disclosed abnormal signals in the middle cerebellar peduncles, brain stem and dentate nuclei in addition to low signals in the caudate and lenticular nuclei, and high signals in the lateral part of the putamen on T2-sequence.(ABSTRACT TRUNCATED AT 250 WORDS)
对5例肝豆状核变性患者进行了随访磁共振成像(MRI)和计算机断层扫描(CT)检查,间隔时间为6至29个月。我们研究了MRI和CT与临床的相关性,以及MRI和CT检查是否有助于评估治疗效果。除1例无症状患者(患者2,患者1的妹妹)外,对4例患者还进行了正电子发射断层扫描(PET)。MRI观察到肌张力障碍与豆状核病变、平稳跟踪眼球运动异常与脑干病变、严重构音障碍/吞咽困难与尾状核和豆状核病变之间分别存在密切关系。在患者4中,间隔18个月的重复MRI显示,随着临床表现的明显改善,T2加权图像上壳核外侧异常高信号降低。在患者3中,其四肢和躯干有严重肌张力障碍,T2加权图像显示豆状核有高信号。该患者在29个月后MRI观察到豆状核高信号明显降低,此时其神经学表现明显改善。患者5有严重的小脑体征,除尾状核和豆状核低信号以及T2序列上壳核外侧高信号外,小脑中间脚、脑干和齿状核也有异常信号。(摘要截短于250字)