Anterior temporal white matter lesions in myotonic dystrophy with intellectual impairment: an MRI and neuropathological study
Anterior temporal white matter lesions in myotonic dystrophy with intellectual impairment: an MRI and neuropathological study
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DOI：
10.1007/s002340050613
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发表时间：
1998-07-01
期刊：
影响因子：
2.8
通讯作者：
Tashiro, K
中科院分区：
文献类型：
--
作者：
Ogata, A;Terae, S;Tashiro, K
我们使用 MRI 和简易精神状态检查 (MMSE) 对 12 名强直性肌营养不良患者进行了研究，以了解特定的 MRI 结果与智力障碍相关。我们还将它们与智力障碍 MD 尸检病例的神经病理学发现进行了比较。 12 名患者中有 8 名发现轻度智力障碍。在 T2 加权和质子密度加权图像上，12 名患者中有 10 名在脑白质中看到高强度区域。其中 7 例发现前颞叶白质病变 (ATWML)；所有七名患者均患有轻度智力障碍（MMSE 22-26），而精神状态正常的四名患者均未患有ATWML。八名智力障碍患者中只有一名未发现白质病变。病理结果显示，除了前颞白质内的异位神经元外，髓鞘和轴突也严重缺失和排列紊乱。双侧 ATWML 可能是 MD 智力障碍的一个因素。回顾性病理学研究提出了 ATWML 与白质局灶性发育不良兼容的可能性。
We studied 12 patients with myotonic dystrophy using MRI and the Mini-mental state examination (MMSE), to see it specific MRI findings were associated with intellectual impairment. We also compared them with the neuropathological findings in an autopsy case of MD with intellectual impairment. Mild intellectual impairment was found in 8 of the 12 patients. On T2-weighted and proton density-weighted images, high-intensity areas were seen in cerebral white matter in 10 of the 12 patients. In seven of these, anterior temporal white-matter lesions (ATWML) were found; all seven had mild intellectual impairment (MMSE 22-26), whereas none of the four patients with normal mentation had ATWML, In only one of the eight patients with intellectual impairment were white-matter lesions not found. Pathological findings were severe loss and disordered arrangement of myelin sheaths and axons in addition to heterotopic neurons within anterior temporal white matter. Bilateral ATWML might be a factor for intellectual impairment in MD. The retrospective pathological study raised the possibility that the ATWML are compatible with focal dysplasia of white matter.