背景:几项单中心研究提出迷走神经(VN)超声用于检测疾病严重程度,自主神经功能障碍,肌萎缩侧索硬化症(ALS)的球表型。然而,由此产生的文献显示出相反的结果,VN超声在ALS中的临床益处留下了相当大的不确定性。
方法:相关研究已确定,直到2024年4月,从各自作者获得的个体患者数据(IPD)与迄今为止未发表的队列(来自慕尼黑)合并。对109例可能或明确的ALS(ElEscorial标准)和可用VN横截面积(CSA)的患者进行了IPD荟萃分析,随着年龄,性别,ALS功能评定量表修订(ALSFRS-R),疾病持续时间,和球表型作为独立变量。
结果:平均年龄为65岁(±12),47%的患者(±12)患有延性肌萎缩侧索硬化症。平均ALSFRS-R为38(±7),平均持续时间为18个月(±18)。VN萎缩非常普遍[左:67%(±5),平均CSA1.6mm2(±0.6);右:78%(±21),平均CSA1.8mm2(±0.7)]。VNCSA与疾病持续时间相关(平均斜率:左-0.01;右-0.01),但没有ALSFRS-R(平均斜率:左0.004;平均斜率:右-0.002)。表型测试Bulbar与非延髓ALS表现不佳(汇总受试者工作特征曲线下面积:左0.496;右0.572).
结论:ALS中的VN萎缩非常普遍,并且与疾病持续时间相关。但不是ALSFRS-R。VNCSA不足以区分球与非球ALS表型。需要进一步的研究来分析VN萎缩之间的联系,自主神经损伤,在ALS中生存。
BACKGROUND: Several single-center studies proposed utility of vagus nerve (VN) ultrasound for detecting disease severity, autonomic dysfunction, and bulbar phenotype in amyotrophic lateral sclerosis (ALS). However, the resulting body of literature shows opposing results, leaving considerable uncertainty on the clinical benefits of VN ultrasound in ALS.
METHODS: Relevant studies were identified up to 04/2024 and individual patient data (IPD) obtained from the respective authors were pooled with a so far unpublished cohort (from Munich). An IPD meta-analysis of 109 patients with probable or definite ALS (El Escorial criteria) and available VN cross-sectional area (CSA) was performed, with age, sex, ALS Functional Rating Scale-revised (ALSFRS-R), disease duration, and bulbar phenotype as independent variables.
RESULTS: Mean age was 65 years (± 12) and 47% of patients (± 12) had bulbar ALS. Mean ALSFRS-R was 38 (± 7), and mean duration was 18 months (± 18). VN atrophy was highly prevalent [left: 67% (± 5), mean CSA 1.6mm2 (± 0.6); right: 78% (± 21), mean CSA 1.8 mm2 (± 0.7)]. VN CSA correlated with disease duration (mean slope: left - 0.01; right - 0.01), but not with ALSFRS-R (mean slope: left 0.004; mean slope: right - 0.002). Test accuracy for phenotyping bulbar vs. non-bulbar ALS was poor (summary receiver operating characteristic area under the curve: left 0.496; right 0.572).
CONCLUSIONS: VN atrophy in ALS is highly prevalent and correlates with disease duration, but not with ALSFRS-R. VN CSA is insufficient to differentiate bulbar from non-bulbar ALS phenotypes. Further studies are warranted to analyze the link between VN atrophy, autonomic impairment, and survival in ALS.