Chipika Rangariroyashe H, Siah We Fong, Shing Stacey Li Hi, Finegan Eoin, McKenna Mary Clare, Christidi Foteini, Chang Kai Ming, Karavasilis Efstratios, Vajda Alice, Hengeveld Jennifer C, Doherty Mark A, Donaghy Colette, Hutchinson Siobhan, McLaughlin Russell L, Hardiman Orla, Bede Peter
Computational Neuroimaging Group, Biomedical Sciences Institute, Trinity College Dublin, Ireland.
First Department of Neurology, Aeginition Hospital, National and Kapodistrian University of Athens, Greece.
Data Brief. 2020 Sep 1;32:106246. doi: 10.1016/j.dib.2020.106246. eCollection 2020 Oct.
A standardised imaging protocol was implemented to evaluate disease burden in specific thalamic and amygdalar nuclei in 133 carefully phenotyped and genotyped motor neuron disease patients. "Switchboard malfunction in motor neuron diseases: selective pathology of thalamic nuclei in amyotrophic lateral sclerosis and primary lateral sclerosis" [1] "Amygdala pathology in amyotrophic lateral sclerosis and primary lateral sclerosis" [2] Raw volumetric data, group comparisons, effect sizes and percentage change are presented. Both ALS and PLS patients exhibited focal thalamus atrophy in ventral lateral and ventral anterior regions revealing extrapyramidal motor degeneration. Reduced accessory basal nucleus and cortical nucleus volumes were noted in the amygdala of negative ALS patients compared to healthy controls. ALS patients carrying the GGGGCC hexanucleotide repeats in exhibited preferential pathology in the mediodorsal-paratenial-reuniens thalamic nuclei and in the lateral nucleus and cortico-amygdaloid transition area of the amygdala. Considerable thalamic atrophy was observed in the sensory nuclei and lateral geniculate region of PLS patients. Our data demonstrate genotype-specific patterns of thalamus and amygdala involvement in ALS and a distinct disease-burden pattern in PLS. The dataset may be utilised for validation purposes, meta-analyses and the interpretation of thalamic and amygdalar profiles from other ALS genotypes.
实施了标准化成像方案，以评估133例经过仔细表型和基因分型的运动神经元病患者特定丘脑和杏仁核中的疾病负担。“运动神经元病中的总机故障：肌萎缩侧索硬化症和原发性侧索硬化症中丘脑核的选择性病理学”[1]“肌萎缩侧索硬化症和原发性侧索硬化症中的杏仁核病理学”[2]呈现了原始体积数据、组间比较、效应量和百分比变化。肌萎缩侧索硬化症（ALS）患者和原发性侧索硬化症（PLS）患者在腹外侧和腹前区均表现出局灶性丘脑萎缩，提示锥体外系运动变性。与健康对照相比，阴性ALS患者杏仁核中的副基底核和皮质核体积减小。携带GGGGCC六核苷酸重复序列的ALS患者在丘脑的内侧背-旁正中-连合核以及杏仁核的外侧核和皮质-杏仁核过渡区表现出优先病理学改变。在PLS患者的感觉核和外侧膝状体区域观察到明显的丘脑萎缩。我们的数据表明，在ALS中丘脑和杏仁核受累存在基因型特异性模式，而在PLS中存在独特的疾病负担模式。该数据集可用于验证目的、荟萃分析以及解释来自其他ALS基因型的丘脑和杏仁核特征。
