Department of Neurology, University of Rostock, Gehlsheimer Str. 20, 18147, Rostock, Germany.
J Neurol. 2012 Sep;259(9):1848-59. doi: 10.1007/s00415-012-6420-y. Epub 2012 Feb 21.
The aim of this work was to investigate white-matter microstructural changes within and outside the corticospinal tract in classical amyotrophic lateral sclerosis (ALS) and in lower motor neuron (LMN) ALS variants by means of diffusion tensor imaging (DTI). We investigated 22 ALS patients and 21 age-matched controls utilizing a whole-brain approach with a 1.5-T scanner for DTI. The patient group was comprised of 15 classical ALS- and seven LMN ALS-variant patients (progressive muscular atrophy, flail arm and flail leg syndrome). Disease severity was measured by the revised version of the functional rating scale. White matter fractional anisotropy (FA) was assessed using tract-based spatial statistics (TBSS) and a region of interest (ROI) approach. We found significant FA reductions in motor and extra-motor cerebral fiber tracts in classical ALS and in the LMN ALS-variant patients compared to controls. The voxel-based TBSS results were confirmed by the ROI findings. The white matter damage correlated with the disease severity in the patient group and was found in a similar distribution, but to a lesser extent, among the LMN ALS-variant subgroup. ALS and LMN ALS variants are multisystem degenerations. DTI shows the potential to determine an earlier diagnosis, particularly in LMN ALS variants. The statistically identical findings of white matter lesions in classical ALS and LMN variants as ascertained by DTI further underline that these variants should be regarded as part of the ALS spectrum.
本研究旨在通过弥散张量成像（DTI），研究经典肌萎缩侧索硬化症（ALS）和下运动神经元（LMN）ALS 变异型中皮质脊髓束内和外的白质微观结构变化。我们利用 1.5T 扫描仪对 22 名 ALS 患者和 21 名年龄匹配的对照者进行了全脑弥散张量成像研究。患者组包括 15 名经典 ALS 患者和 7 名 LMN ALS 变异型患者（进行性肌萎缩症、挥舞臂和挥舞腿综合征）。疾病严重程度采用功能评定量表修订版进行评估。采用基于束的空间统计学（TBSS）和感兴趣区（ROI）方法评估白质各向异性分数（FA）。与对照组相比，我们发现经典 ALS 和 LMN ALS 变异型患者的运动和运动外脑纤维束的 FA 明显降低。基于体素的 TBSS 结果得到 ROI 结果的证实。在患者组中，白质损伤与疾病严重程度相关，且在 LMN ALS 变异型亚组中，白质损伤的分布相似，但程度较轻。ALS 和 LMN ALS 变异型是多系统退行性疾病。DTI 具有确定早期诊断的潜力，特别是在 LMN ALS 变异型中。DTI 确定的经典 ALS 和 LMN 变异型中白质病变的统计学相同发现进一步强调，这些变异型应被视为 ALS 谱的一部分。
