Cerebral cortical and white matter lesions in amyotrophic lateral sclerosis with dementia: Correlation with MR and pathologic examinations
Cerebral cortical and white matter lesions in amyotrophic lateral sclerosis with dementia: Correlation with MR and pathologic examinations
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DOI：
10.3174/ajnr.a0605
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发表时间：
2007-09-01
影响因子：
3.5
通讯作者：
Ogawa, T.
中科院分区：
文献类型：
--
作者：
Matsusue, E.;Sugihara, S.;Ogawa, T.
背景和目的：肌萎缩侧索硬化伴痴呆（ALSD）是一种进行性神经退行性疾病，其临床特征在于运动神经元症状和痴呆，病理特征在于脑和脊髓的运动神经元变性以及额叶和/或颞叶萎缩。到目前为止，还没有关于ALSD的MR图像与组织学表现的相关性研究。材料与方法：对3例ALSD. Results尸检病例的T2加权像与组织学检查结果进行对比分析。结果：生前MRI显示额叶和颞叶萎缩，颞叶内腹侧不对称。1例颞叶皮质下白色质的内腹侧部分可见微弱的线性T2高信号。尸检T2加权图像显示，在每一个案件的腹内侧颞叶线性皮质下高信号。组织学上，皮质萎缩的MR图像表现为海绵状变化，神经元丢失和胶质增生，特别是在浅表层和线性皮质下高信号的T2加权图像显示变性和胶质增生的情况下，在每种情况下。结论：额叶和颞叶萎缩伴颞叶前内侧皮质下线状高信号MRI对ALSD的诊断有重要价值。
BACKGROUND AND PURPOSE: Amyotrophic lateral sclerosis with dementia (ALSD) is a progressive neurodegenerative disorder, characterized clinically by motor neuron symptoms and dementia, and pathologically by degeneration of the motor neurons of the brain and spinal cord as well as atrophy of the frontal and/or temporal lobes. So far, there has been no study on the correlation of MR images with histologic findings in ALSD. We studied the correlation of antemortem and postmortem T2-weighted MR images with histologic findings in autopsy-proved cases of ALSD.MATERIALS AND METHODS: Antemortem and postmortem T2-weighted images were compared with histologic findings in 3 autopsy-proved cases of ALSD.RESULTS: Antemortem MR images showed atrophy of the frontal and temporal lobes, which were asymmetric in the medial-ventral part of the temporal lobe. Faint linear T2-hyperintensity was seen in the medial-ventral part of the temporal subcortical white matter in 1 case. Postmortem T2-weighted images showed linear subcortical hyperintensity in the ventral-medial temporal lobe in each case. Histologically, cortical atrophy on MR images showed spongiform change with neuronal loss and gliosis especially in the superficial layers and linear subcortical hyperintensity on T2-weighted images showed degeneration and gliosis in each case. These findings are characteristic histologic changes of ALSD.CONCLUSION: MR imaging of atrophy of the frontal and temporal lobes with linear subcortical hyper-intensities in the anteromedial temporal lobe is useful for diagnosis of ALSD.