Stikvoort García Diederik J L, van den Berg Leonard H, Sleutjes Boudewijn T H M, Goedee H Stephan
Department of Neurology, Brain Center Utrecht, University Medical Center Utrecht, Utrecht, The Netherlands.
Muscle Nerve. 2025 Apr;71(4):680-684. doi: 10.1002/mus.28326. Epub 2025 Jan 20.
INTRODUCTION/AIMS: Reduced nerve sizes obtained by nerve ultrasound (NUS) have been proposed as a potential diagnostic marker for amyotrophic lateral sclerosis (ALS). However, prospective studies evaluating patients with suspected ALS are currently lacking. We, therefore, evaluated the diagnostic accuracy of a standardized NUS protocol in a large sample of suspected ALS patients.
We prospectively recruited 193 patients with suspected ALS, all of whom underwent the relevant ancillary tests. They also underwent a standardized NUS protocol, evaluating median nerve cross-sectional area (CSA) at upper arm, forearm and wrist. Additionally, we selected, retrospectively, a random sample of incident patients with multifocal motor neuropathy (MMN, n = 42). We determined diagnostic accuracy using receiver operating characteristic (ROC) analysis.
Ultimately, 143/193 patients received a final diagnosis of ALS, at a median disease duration of 10 months. Fifty patients were classified as non-ALS. Diagnostic yield of NUS to distinguish between patients with and without ALS was low (highest area under the curve (AUC) at the wrist: 0.57). In contrast, abnormal nerve sizes accurately discriminated MMN from patients with ALS, with AUCs ranging from 0.65 at the wrist to 0.86 at the upper arm.
Our study shows that reductions in nerve size are unlikely to have diagnostic utility during routine evaluation of suspected patients with ALS. However, when the differential diagnosis includes both ALS and MMN, median nerve size demonstrates high diagnostic accuracy.
引言/目的：神经超声（NUS）测得的神经尺寸减小已被提议作为肌萎缩侧索硬化症（ALS）的一种潜在诊断标志物。然而，目前缺乏对疑似ALS患者进行评估的前瞻性研究。因此，我们评估了标准化NUS方案在大量疑似ALS患者样本中的诊断准确性。
我们前瞻性招募了193例疑似ALS患者，所有患者均接受了相关辅助检查。他们还接受了标准化NUS方案，评估上臂、前臂和腕部的正中神经横截面积（CSA）。此外，我们回顾性随机抽取了多灶性运动神经病（MMN，n = 42）的新发患者样本。我们使用受试者操作特征（ROC）分析来确定诊断准确性。
最终，143/193例患者最终被诊断为ALS，疾病持续时间中位数为10个月。50例患者被归类为非ALS。NUS区分ALS患者和非ALS患者的诊断率较低（腕部曲线下面积（AUC）最高：0.57）。相比之下，神经尺寸异常能准确区分MMN患者与ALS患者，AUC范围从腕部的0.65到上臂的0.86。
我们的研究表明，在对疑似ALS患者进行常规评估时，神经尺寸减小不太可能具有诊断价值。然而，当鉴别诊断包括ALS和MMN时，正中神经尺寸显示出较高的诊断准确性。
