Troncoso M, Badilla L, Bravo E, Miranda M, Gajewski C, Barrios A, Villagra R
Servicio de Neurología Infantil, Hospital Clínico San Borja Arriarán, Santiago-Chile.
Rev Med Chil. 1998 Jan;126(1):81-7.
Wilson disease is an inborn error of copper metabolism that has neurological and hepatic manifestations. We report a 13 years old girl and a 12 years old boy with Wilson disease. In both patient, brain computed tomography and magnetic resonance imaging showed marked involvement of basal ganglia and other deep gray nuclei. Considering that this is a treatable disease, it should be included in the differential diagnosis of the so called "striatal necrosis of childhood".
威尔逊病是一种铜代谢的先天性缺陷疾病，具有神经和肝脏方面的表现。我们报告了一名13岁女孩和一名12岁男孩患有威尔逊病。两名患者的脑部计算机断层扫描和磁共振成像均显示基底神经节和其他深部灰质核有明显受累。鉴于这是一种可治疗的疾病，在所谓“儿童纹状体坏死”的鉴别诊断中应予以考虑。
