中文摘要：

目的应用静息态功能磁共振成像（＊fMRI）分数低频振幅（fractionalamplitudeoflow—frequencyfluctuation，fAl。FF）方法对肌萎缩侧索硬化（amyotrophiclateralsclerosis，AI。s）患者基线脑活动变化进行研究。方法利用SiemensTrioTim3．0TMRI对AI．S组和正常对照组（各12例）分别进行静息态fMRI扫描。静息态fMRI数据处理助手（DPARSF）基于Matlab2009a平台进行数据处理，应用fAI。FF统计方法计算并对比2组fALFF改变的脑区。并分析存在显著差异脑区的fALFF值与临床参数如肌萎缩侧索硬化功能等级量表（ALSFRS—r）、病程、疾病预后比值的相关性。结果相对正常对照组，ALs患者fALFF值降低区域主要位于左侧前扣带回、左侧额上中回、左侧顶下叶，fALFF降低有统计学意义（P〈70．001，AlphaSim校正），并未发现fAI，FF显著增高的脑区。在ALS患者中，左侧前扣带回、左侧额上中回分别和患者的病程呈负相关（分别为r=0．690，P=：0．013；r=0．710，P=0．010），差异脑区fALFF值和患者ALSFRS-r及疾病预后比值之间并无显著的相关性（P〉0．05）。结论ALS患者静息态下脑功能区fAI。FF值存在显著变化，表明ALS是一种多系统性疾病。左侧前扣带回、左侧额上中回fALFF值可作为ALS患者疾病累进的定量标志。

英文摘要：

Objective To investigate the baseline brain activity level in patients with amyotrophic lateral sclerosis （ALS} by frac- tional amplitude of low-frequency fluctuation{ fAI.FF）based on resting state functional MRI （rs-fMRI）. Methods Twelve patients with AI.S （AI.S group） and 12 age-, and sex-matched healthy controls （HC group） were examined by resting-state fMR1 on Siemens Trio Tim 3. 0T. The data preprocessing and processing was performed using data processing assistant for resting-state fMRI （DPARSF） based on Matlab 2009a, fAI.FF values of the two groups were calculated and compared between the AI.S group and the control group. In ALS group, the fAI.FF value in different region between two groups correlated with the revised amyotrophic later- al sclerosis functional rating scale （ALSFRS-r）, rate of disease progression and disease duration. Results Compared with the healthy controls, the patients with ALS had significantly decreased fAI.FF values in the left superior frontal gyrus （BA9, 10）, left anterior cingulate cortex （BA32, 10} and left Inferior parietal lobule （BA 19}, with statistically significant differences （P（ 0.01, corrected by AlphaSim}, repectively; whereas no region showing increased fALFF values in patients with AI.S. We found a negative correlations between the means fAI.FF values in left superior frontal gyrus and left anterior cingulate cortex and disease duration in the ALS group. However, there was no significant correlation between the disease severity {ALSFRS scores）, the rate of disease progression and the mean fALFF in AI.S group. Conclusion This rs-fMRI study suggests that the alteration brain activity in the fALFF contributes to the understanding of the pathophysiologieal mechanism of AI.S patients,and supporting the concept that ALS is a multisystem disorder. This study indicates that the [ALFF value in the left superior frontal gyms and left anterior cingulate cortex may be a quantitative marker in progress of ALS disease.