Institute of Biomedical Technologies, National Research Council, Segrate, Italy.
Division of Neuroscience, San Raffaele Scientific Institute, Milano, Italy; Department of Clinical Neurosciences, IRCCS San Raffaele Turro, Milano, Italy.
Neurobiol Aging. 2018 Feb;62:95-104. doi: 10.1016/j.neurobiolaging.2017.09.019. Epub 2017 Sep 28.
The continuum hypothesis linking the behavioral variant of frontotemporal dementia (bvFTD) and amyotrophic lateral sclerosis (ALS) is supported by clinical, pathological, genetic, and neuroimaging evidence. In the present multimodal magnetic resonance study, we characterized the site and extent of shared neurostructural changes in gray and white matter in 20 bvFTD and 19 ALS patients without dementia. We found an overlap of macrostructural and microstructural damage in both patient groups compared with healthy controls, involving the right orbital and the bilateral anterior cingulate cortices, the corticospinal tract and corpus callosum. The quantification of gray and white matter damage within the areas of shared alterations highlighted a higher degree of atrophy in orbitofrontal and frontomedial regions in patients with more severe executive and/or behavioral symptoms, and a higher degree of degeneration in the motor pathway in patients with more severe motor neuron disorders. Our finding provides additional evidence confirming the FTD-ALS continuum hypothesis and supports the notion of a bimodal but convergent pattern of neurostructural changes characterizing bvFTD and ALS.
将额颞叶痴呆（bvFTD）和肌萎缩侧索硬化症（ALS）联系起来的连续体假说得到了临床、病理、遗传和神经影像学证据的支持。在本项多模态磁共振研究中，我们对 20 名无痴呆的 bvFTD 和 19 名 ALS 患者的灰质和白质的共享神经结构变化的部位和程度进行了特征描述。与健康对照组相比，我们发现两组患者的宏观和微观结构损伤均存在重叠，涉及右侧眶额和双侧前扣带皮质、皮质脊髓束和胼胝体。在共享改变区域内对灰质和白质损伤的定量分析突出显示，执行和/或行为症状更严重的患者额眶和额内侧区域的萎缩程度更高，运动神经元疾病更严重的患者运动通路的退化程度更高。我们的发现提供了额外的证据，证实了 FTD-ALS 连续体假说，并支持了以 bvFTD 和 ALS 为特征的双模态但趋同的神经结构变化模式的概念。
