Brain MRI features of congenital- and adult-form myotonic dystrophy type 1: case-control study
Brain MRI features of congenital- and adult-form myotonic dystrophy type 1: case-control study
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DOI：
10.1016/s0960-8966(01)00324-8
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发表时间：
2002-06-01
影响因子：
2.8
通讯作者：
Tedeschi, G
中科院分区：
文献类型：
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作者：
Di Costanzo, A;Di Salle, F;Tedeschi, G
为了比较和表征先天性和成人型 1 型强直性肌营养不良症的大脑磁共振成像 (MRI)，我们评估了 5 名先天性 1 型肌营养不良症患者、10 名年龄匹配和 10 名病程匹配的成人 1 型肌营养不良症患者以及 20 名年龄匹配的健康志愿者。通过脑室与脑的比例、参照标准图像的三角区后上白质信号强度以及半定量方法的白质病变来评估脑室扩大。先天性营养不良1型患者的MRI表现为脑室扩大、三角区后上白质中度/重度高信号，与年龄无关。成人型 1 型营养不良症的 MRI 与病程密切相关，并且年龄匹配患者的正常表现（颞极白质病变除外）和病程匹配患者的脑室扩大伴白质高信号之间存在差异。这些结果表明，I 型强直性肌营养不良症的 MRI 异常起源对于先天性肌营养不良来说主要是发育性的，对于成人型肌营养不良来说主要是退行性的。 (C) 2002 Elsevier Science B.V. 保留所有权利。
To compare and characterize the magnetic resonance imaging (MRI) of brain in the congenital and adult form of myotonic dystrophy type 1, we evaluated five patients with congenital dystrophy type 1, 10 age- and 10 disease duration-matched patients with adult-form dystrophy type 1 and 20 age-matched healthy volunteers. The ventricular enlargement was evaluated by the ventricular:brain ratio, the signal intensity of white matter posterosuperior to trigones by reference to standard images and the white matter lesions by a semiquantitative method. In the congenital dystrophy type 1, MRI was characterized by ventriculomegaly and moderate/severe hyperintensity of white matter posterosuperior to trigones, which showed no correlation with the age. MRI in the adult-form dystrophy type 1 was strictly related to disease duration and varied between normal findings, except for temporo-polar white matter lesions, in age-matched patients and ventriculomegaly with white matter hyperintensities in disease duration-matched patients. These results suggest that the origin of MRI abnormalities in myotonic dystrophy type I is mainly developmental for the congenital form and mainly degenerative for the adult form. (C) 2002 Elsevier Science B.V. All rights reserved.