Department of Radiology, NewYork-Presbyterian Hospital - Weill Cornell Medicine, New York, NY, USA.
Department of Radiology, Stony Brook Medicine, Stony Brook, NY, USA.
Clin Imaging. 2021 Jul;75:125-130. doi: 10.1016/j.clinimag.2020.12.018. Epub 2021 Jan 4.
Accurate and timely diagnosis of amyotrophic lateral sclerosis (ALS) is a diagnostic challenge given the lack of specific diagnostic and imaging biomarkers as well as the significant clinic overlap with mimic syndromes. We hypothesize that MR quantitative susceptibility mapping (QSM) can help differentiate ALS from mimic diagnoses.
In a blinded retrospective study of MRIs with QSM from 2015 to 2018, we compared motor cortex susceptibility along the hand and face homunculi in ALS patients and patients with similar clinical presentations. Inclusion required a confirmed ALS or a mimic diagnosis. Comparative groups included age-matched patients with MRIs performed for non-motor neuron symptoms that were reported as normal or demonstrated leukoaraiosis. Quantitative susceptibility values were compared with ANOVA and Tukey-Kramer (post-hoc). ROC analysis and Youden's index were used to identify optimal cutoff values.
Fifty ALS, 35 mimic, and 70 non-motor neuron symptom patients (35 normal, 35 leukoaraiosis) were included. Hand and face homunculus mean susceptibility values were significantly higher in the ALS group compared to the mimic (p=0.001, p=0.004), leukoaraiosis (p<0.001, p=0.003), and normal (p<0.001, p<0.001) groups. ROC curve analysis comparing ALS to mimics resulted in an area under the curve of 0.71 and 0.67 for the hand and face homunculus measurements, respectively. In differentiating ALS from mimics, Youden's index showed 100% specificity and 36% sensitivity for hand homunculus measurements.
QSM has diagnostic potential in the assessment of suspected ALS patients, demonstrating very high specificity in differentiating ALS from mimic diagnoses.
由于缺乏特定的诊断和成像生物标志物，以及与模仿综合征的显著临床重叠，肌萎缩侧索硬化症（ALS）的准确和及时诊断是一个诊断挑战。我们假设磁共振定量磁化率（QSM）可以帮助区分 ALS 与模仿诊断。
在一项 2015 年至 2018 年的 QSM 磁共振成像的盲法回顾性研究中，我们比较了 ALS 患者和具有相似临床表现的患者的运动皮层磁化率沿手和面部同源物。纳入标准需要确认的 ALS 或模仿诊断。对照组包括年龄匹配的因非运动神经元症状进行 MRI 检查的患者，这些患者的 MRI 结果报告为正常或显示出脑白质疏松症。定量磁化率值通过方差分析和 Tukey-Kramer（事后）进行比较。ROC 分析和 Youden 指数用于确定最佳截断值。
共纳入 50 例 ALS 患者、35 例模仿诊断患者和 70 例非运动神经元症状患者（35 例正常，35 例脑白质疏松症）。与模仿组（p=0.001，p=0.004）、脑白质疏松症组（p<0.001，p=0.003）和正常组（p<0.001，p<0.001）相比，手和面部同源物的平均磁化率值在手和面部同源物测量中，ALS 组明显更高。比较 ALS 与模仿组的 ROC 曲线分析结果分别为 0.71 和 0.67。在手同源物测量中，区分 ALS 与模仿组，Youden 指数显示出 100%的特异性和 36%的敏感性。
QSM 在评估疑似 ALS 患者方面具有诊断潜力，在手和面部同源物测量中，特异性非常高，可区分 ALS 与模仿诊断。
