Temporal lobe studies in motor neuron disease overwhelmingly focus on white matter alterations and cortical grey matter atrophy. Reports on amygdala involvement are conflicting and the amygdala is typically evaluated as single structure despite consisting of several functionally and cytologically distinct nuclei. A prospective, single-centre, neuroimaging study was undertaken to comprehensively characterise amygdala pathology in 100 genetically-stratified ALS patients, 33 patients with PLS and 117 healthy controls. The amygdala was segmented into groups of nuclei using a Bayesian parcellation algorithm based on a probabilistic atlas and shape deformations were additionally assessed by vertex analyses. The accessory basal nucleus (p = .021) and the cortical nucleus (p = .022) showed significant volume reductions in C9orf72 negative ALS patients compared to controls. The lateral nucleus (p = .043) and the cortico-amygdaloid transition (p = .024) were preferentially affected in C9orf72 hexanucleotide carriers. A trend of total volume reduction was identified in C9orf72 positive ALS patients (p = .055) which was also captured in inferior-medial shape deformations on vertex analyses. Our findings highlight that the amygdala is affected in ALS and our study demonstrates the selective involvement of specific nuclei as opposed to global atrophy. The genotype-specific patterns of amygdala involvement identified by this study are consistent with the growing literature of extra-motor clinical features. Mesial temporal lobe pathology in ALS is not limited to hippocampal pathology but, as a key hub of the limbic system, the amygdala is also affected in ALS.

中文翻译：

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肌萎缩侧索硬化和原发性侧索硬化的杏仁核病理学

运动神经元疾病的颞叶研究主要集中在白质改变和皮质灰质萎缩上。关于杏仁核受累的报告是相互矛盾的，尽管由几个功能和细胞学上不同的细胞核组成，但杏仁核通常被评估为单一结构。进行了一项前瞻性、单中心、神经影像学研究，以全面表征 100 名遗传分层 ALS 患者、33 名 PLS 患者和 117 名健康对照的杏仁核病理。使用基于概率图谱的贝叶斯分割算法将杏仁核分割成核组，并通过顶点分析额外评估形状变形。辅助基底核 (p = .021) 和皮质核 (p = . 022) 显示与对照组相比，C9orf72 阴性 ALS 患者的体积显着减少。外侧核 (p = .043) 和皮质-杏仁核转变 (p = .024) 在 C9orf72 六核苷酸载体中优先受到影响。在 C9orf72 阳性 ALS 患者中发现了总体积减少的趋势（p = .055），这在顶点分析的下内侧形状变形中也被捕获。我们的研究结果强调杏仁核在 ALS 中受到影响，我们的研究表明特定细胞核的选择性参与而不是整体萎缩。本研究确定的杏仁核受累的基因型特异性模式与越来越多的运动外临床特征文献一致。ALS 的内侧颞叶病理不仅限于海马病理，而且作为边缘系统的关键枢纽，