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首页> 外文期刊>The European Journal of Neuroscience >Magnetic resonance imaging reveals neuronal degeneration in the brainstem of the superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis.
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Magnetic resonance imaging reveals neuronal degeneration in the brainstem of the superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis.

机译：磁共振成像揭示肌萎缩性侧索硬化的超氧化物歧化酶1转基因小鼠模型的脑干中的神经元变性。

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摘要

Abstract Magnetic resonance imaging (MRI) is becoming the preferred neuroimaging modality for the diagnosis of human amyotrophic lateral sclerosis (ALS). A useful animal model of ALS is the superoxide dismutase 1(G93A G1H) transgenic mouse, which shows many of the clinico-pathological features of the human condition. We have employed a 4.7-Tesla MRI instrument to determine whether a noninvasive imaging approach can reveal pathological changes in the nervous system of this animal model. Our T(2)-weighted MRI revealed consistent changes in brain and brainstem of these mice. Hyperintensities, indicative of neuropathology, were observed in several areas including the nucleus ambiguus, facial nucleus, trigeminal motor nucleus, rostroventrolateral reticular nucleus, lateral paragigantocellular nucleus and the substantia nigra. Histology analysis including neuronal counts of the imaged brains confirmed the T(2)-weighted MRI findings. Enlarged ventricles and hypointense striations, indicative of global atrophy, were also observed in the brain and cerebellum. This atrophy was confirmed by fresh brain weight data. The extensive global degeneration involving multiple structures suggests a multisystem disease that is similar to human ALS.
机译：摘要磁共振成像（MRI）正成为诊断人类肌萎缩性侧索硬化症（ALS）的首选神经影像学方法。有用的ALS动物模型是超氧化物歧化酶1（G93A G1H）转基因小鼠，它显示出人类疾病的许多临床病理特征。我们采用了4.7-Tesla MRI仪器来确定无创成像方法是否可以揭示该动物模型神经系统的病理变化。我们的T（2）加权MRI揭示了这些小鼠的大脑和脑干的一致变化。在几个区域观察到了指示神经病理学的高信号，包括歧核，面核，三叉神经运动核，腹侧外侧网状核，旁旁巨细胞核和黑质。组织学分析，包括成像大脑的神经元计数，证实了T（2）加权MRI结果。在脑和小脑中也观察到脑室增大和低水平横纹，这表明整体萎缩。新鲜的脑重量数据证实了这种萎缩。涉及多个结构的广泛的全球变性提示与人类ALS相似的多系统疾病。

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