肝豆状核变性的脑MRI诊断

MRI of the Brain in Hepatolenticular Degeneration

作者对８例临床确诊的肝豆状核变性脑ＭＲＩ进行了分析。结果表明异常信号分布广泛并且对称出现，以基底节、丘脑和脑干为著。病变形态及信号较具特点。Ｔ＿２加权像高信号较常见，其信号基础为继发于铜沉积的水肿、胶质增生或空腔，Ｔ＿２加权像低信号为本病特征性改变，系铜的顺磁性作用造成Ｔ＿２弛豫时间缩短所致，它的出现对病程判断很有帮助。对ＭＲＩ与临床表现的关系也作了初步探讨。作者认为ＭＲＩ对肝豆状核变性的早期诊断、疗效观察以及评价预后有很大意义。

MR findings in eight patients with clinically proved hepatolenticular degeneration(Wilson disease) were retrospectively analyzed. The results demonstrated that the abnormalities oc-curred symmetrically and extensively, with predominant involvement of the basal ganglia, thalamus,and brain stem. High signal intensities were commonly seen on T_2-weighted images, suggestive of copper induced edema, gliosis or cavitation. Hypointensities were characteristic on T_2-weighted se-quences, representing excessive copper deposition and its paramagnetic T_2-shortening effect, and was important in assessing progress of disease. Preliminary studies were also made on the correlation of MR imagings with the clinical manifestations. The authors conclude that MRI is useful for early diag-nosis of hepatolenticular degeneration, as well as in evaluating therapy and assessing prognosis.