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[肝豆状核变性的脑磁共振成像]

[Cerebral magnetic resonance in Wilson's disease].

作者信息

Amato C, Bisceglie P, Moschini M

机构信息

Servizio di Radiologia, IRCCS Oasi, Troina, Enna.

出版信息

Radiol Med. 1994 Dec;88(6):752-7.

PMID:7878232
Abstract

The authors describe the typical and atypical MR findings of brain abnormalities in Wilson's disease in three patients affected with severe neurologic disturbances; a low-field MR unit was used. Radiologic findings included atrophic changes and focal lesions. Two patients had basal ganglia, brain stem and dentate alterations; lesions in the corpus callosum (a site not yet described in Wilson's disease) were seen. The third case had putaminal lesions which improved after penicillamine therapy. Cerebral abnormalities were demonstrated as areas of increased signal on T2-weighted images; T1 and T2 shortening due to magnetic susceptibility phenomena was not seen. Two major MR features were observed: high hyperintensity and peripheral location of putaminal lesions and sparing of the medial thalami in diffuse basal ganglia involvement.

摘要

作者描述了3例患有严重神经功能障碍的威尔逊病患者脑部异常的典型和非典型磁共振成像(MR)表现;使用的是低场MR设备。放射学表现包括萎缩性改变和局灶性病变。2例患者出现基底节、脑干和齿状核改变;观察到胼胝体病变(威尔逊病中尚未描述的部位)。第3例患者有壳核病变,青霉胺治疗后有所改善。脑异常在T2加权图像上表现为信号增强区域;未观察到由于磁敏感性现象导致的T1和T2缩短。观察到两个主要的MR特征:壳核病变的高信号强度和外周位置,以及弥漫性基底节受累时内侧丘脑未受累。

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