Yousaf Muhammad, Kumar Manoj, Ramakrishnaiah Raghu, Vanhemert Rudy, Angtuaco Edgardo
Radiol Case Rep. 2015 Dec 7;4(3):312. doi: 10.2484/rcr.v4i3.312. eCollection 2009.
Wilson's disease is an inborn error of copper metabolism characterized by inability of the liver to excrete copper into the bile, with excessive deposition of copper primarily in the liver and in the brain. The lentiform nuclei are involved, most often followed by involvement of the thalami, the pons, midbrain, superior and inferior cerebellar peduncles, and the cerebellar nuclei. Predominant involvement of the thalami and brainstem with no significant involvement of the lentiform nuclei is not common. We present a case of Wilson's disease with minimal involvement of the lentiform nuclei, with marked lesions involving the thalami, midbrain, and pons.
威尔逊病是一种铜代谢的先天性缺陷疾病，其特征是肝脏无法将铜排泄到胆汁中，导致铜主要在肝脏和大脑中过度沉积。豆状核会受到影响，最常见的是随后丘脑、脑桥、中脑、小脑上下脚和小脑核也会受到影响。丘脑和脑干主要受累而豆状核无明显受累的情况并不常见。我们报告一例威尔逊病患者，其豆状核受累程度轻微，但丘脑、中脑和脑桥有明显病变。
