Department of Electrical and Electronics Engineering, Birla Institute of Technology and Science Pilani, Hyderabad Campus, Hyderabad, India.
Department of Biomedical Engineering, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA.
Eur J Neurol. 2020 Oct;27(10):1904-1912. doi: 10.1111/ene.14332. Epub 2020 Jun 27.
Amyotrophic lateral sclerosis (ALS) is a motor neuron disorder, although extra-motor degeneration is well recognized, especially in frontotemporal regions manifested as ALS with frontotemporal dementia (ALS-FTD). Previous neuroimaging studies of the brains of ALS-FTD patients have measured abnormalities of either grey matter (GM) or white matter (WM) structures but not of both together. Therefore, the aim was to evaluate both GM and WM in the same ALS-FTD patient using functional and structural neuroimaging. By doing so, insights could be gained into whether neurodegeneration in ALS-FTD is primarily a neuronopathy or axonopathy.
After high-resolution brain 2-deoxy-2-[ F]fluoro-D-glucose ( F-FDG) positron emission tomography (PET) and magnetic resonance imaging (MRI) scans were obtained in ALS-FTD patients and in age- and sex-matched neurological controls, changes in metabolic rate, cortical thickness (CT) and WM network analysis using graph theory were analyzed.
Significant reductions in F-FDG PET metabolism, CT and WM connections were observed in motor and extra-motor brain regions of ALS-FTD patients compared to controls. Both CT and underlying WM networks were abnormal in frontal, temporal, parietal and occipital lobes of ALS-FTD patients with 86 of 90 brain regions showing reductions of CT.
Abnormalities in significantly fewer WM networks underlying the affected cortical regions suggest that neurodegeneration in brains of ALS-FTD patients is primarily a 'neuronopathy' rather than an 'axonopathy.'
肌萎缩侧索硬化症（ALS）是一种运动神经元疾病，尽管已经认识到运动神经元以外的变性，特别是在额颞叶区域表现为肌萎缩侧索硬化症伴额颞叶痴呆（ALS-FTD）。以前对 ALS-FTD 患者大脑的神经影像学研究已经测量了灰质（GM）或白质（WM）结构的异常，但没有同时测量两者。因此，目的是使用功能和结构神经影像学评估同一 ALS-FTD 患者的 GM 和 WM。通过这样做，可以深入了解 ALS-FTD 中的神经退行性变是主要是神经元病还是轴突病。
在 ALS-FTD 患者和年龄、性别匹配的神经科对照组中获得高分辨率脑 2-脱氧-2-[F]氟-D-葡萄糖（F-FDG）正电子发射断层扫描（PET）和磁共振成像（MRI）扫描后，分析代谢率、皮质厚度（CT）和使用图论进行 WM 网络分析的变化。
与对照组相比，ALS-FTD 患者的运动和运动外脑区的 F-FDG PET 代谢、CT 和 WM 连接均明显减少。ALS-FTD 患者的额、颞、顶和枕叶的 CT 和潜在的 WM 网络均异常，90 个脑区中有 86 个显示 CT 减少。
受影响皮质区域下 WM 网络的异常明显较少，表明 ALS-FTD 患者大脑中的神经退行性变主要是“神经元病”而不是“轴突病”。
