Department of Neurology With Friedrich Baur Institute, LMU University Hospital, LMU Munich, Munich, Germany.
Department of Neurology, Leipzig University Hospital, Leipzig, Germany.
J Neurol. 2024 Sep;271(9):6255-6263. doi: 10.1007/s00415-024-12601-z. Epub 2024 Jul 31.
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up to 04/2024 and individual patient data (IPD) obtained from the respective authors were pooled with a so far unpublished cohort (from Munich).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 1.6mm (± 0.6); right: 78% (± 21), mean CSA 1.8 mm (± 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). CONCLUSION: 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.
背景:几项单中心研究提出,迷走神经(VN)超声可用于检测肌萎缩侧索硬化症(ALS)的疾病严重程度、自主神经功能障碍和球部表型。然而,现有的研究结果相互矛盾,使得 VN 超声在 ALS 中的临床获益存在相当大的不确定性。
方法:截至 2024 年 4 月,检索到相关研究,并从各自的作者那里获取了个体患者数据(IPD),并与一个尚未发表的队列(来自慕尼黑)进行了汇总。对 109 例可能或明确的 ALS(埃尔埃斯科里亚尔标准)患者的 IPD 进行了 VN 横截面面积(CSA)的荟萃分析,年龄、性别、肌萎缩侧索硬化功能评定量表修订版(ALSFRS-R)、疾病持续时间和球部表型为独立变量。
结果:平均年龄为 65 岁(±12),47%的患者(±12)有球部 ALS。平均 ALSFRS-R 为 38(±7),平均病程为 18 个月(±18)。VN 萎缩非常普遍[左侧:67%(±5),平均 CSA 为 1.6mm(±0.6);右侧:78%(±21),平均 CSA 为 1.8mm(±0.7)]。VN CSA 与疾病持续时间相关(平均斜率:左侧 -0.01;右侧 -0.01),但与 ALSFRS-R 无关(平均斜率:左侧 0.004;平均斜率:右侧 -0.002)。用于表型鉴定球部与非球部 ALS 的测试准确性较差(汇总受试者工作特征曲线下面积:左侧 0.496;右侧 0.572)。
结论:ALS 中的 VN 萎缩非常普遍,与疾病持续时间相关,但与 ALSFRS-R 无关。VN CSA 不足以区分球部与非球部 ALS 表型。需要进一步研究来分析 VN 萎缩、自主神经损伤与 ALS 生存之间的联系。
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