【摘要】 目的　描述脑内灰质核团铁、铜、锰和钙沉积的MRI表现 ,结合文献讨论其发生机制及其病理改变。方法　回顾性分析Hallervorden Spatz病、Wilson病、肝脑变性以及各种原因引起的钙在脑内灰质核团沉积的病例共 38例的MRI表现。结果　Hallervorden Spatz病表现为双侧苍白球和黑质T2WI明显的低信号 ,并可见苍白球的前内侧对称出现的点状高信号 (虎眼征 ) ;Wilson病 4例均表现为双侧壳核、中脑出现对称性的T2WI高信号 ,其中 1例还可见双侧苍白球对称的T2WI低信号 ;肝脑变性表现为双侧苍白球 (2 7/ 2 9) ,壳核 (6 / 2 9) ,垂体前叶 (13/ 2 9) ,中脑红核周围 (18/ 2 9)等部位出现高信号 ;特发性钙化所致钙在脑内灰质核团沉积表现为基底节、丘脑、齿状核对称出现的等和短T1信号。结论　脑内灰质核团的异常信号有其特定的病因学和病理学基础及相应的MRI表现 ,结合临床表现及实验室检查 ,可以做出正确的诊断。更多还原

【Abstract】 Purpose To describe the MRI features of iron, copper, manganese and calcium deposition in gray matter nuclei. Methods MR imaging of 38 cases with gray matter nuclei abnormal signal intensity including 4 cases of Wilson disease, 1 case of Hallervorden Spatz disease, 29 cases of acquired hepatocerebral degeneration (AHCD), and 2 cases of idiopathic calcification, was studied with the review of literatures. Results Hallervorden Spatz disease presented low signal intensity in T2WI in bilateral globus pallidum and substantia nigra, and an anteromedial area of high signal intensity (eye of the tiger sign). Wilson disease showed signal increase in bilateral putamen, red nucleus, and substantia nigra in T2WI. One case also demonstrated low signal intensity in the bilateral globus pallidum. AHCD demonstrated increased signal intensity in the globus pallidus (27/29), putamen (6/29), anterior pituitary (13/29), and mesencephalon surrounding red nucleus (18/29). Idiopathic calcification can also demonstrated increased signal intensity in T1WI in the bilateral basal ganglia, thalamus, and dentate nuclei. Conclusion Gray mater nuclei abnormal signal intensity has specific etiology and pathology and correlative MRI features. Combined with clinic data, MRI can make correct diagnosis in these kinds of diseases.更多还原