Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
Clin Neurophysiol. 2020 Aug;131(8):2017-2022. doi: 10.1016/j.clinph.2020.04.161. Epub 2020 May 21.
To verify whether the finding of denervation activity on EMG at the time of diagnosis has a prognostic value in amyotrophic lateral sclerosis (ALS).
We retrospectively studied all the patients discharged with a diagnosis of ALS between January 2009 and January 2017. 92 patients met the inclusion criteria. We mainly verified three prognostic targets:All EMG examinations were reviewed and a denervation score (DS) was calculated. The association of DS with clinical milestones was analysed, adjusting for disease duration, age , sex, and clinical phenotype.
We found a significant association between bulbar DS and time to NIV/tracheostomy (HR: 3.34, 95% CI: 1.49 to 7.48, p = 0.002) and with survival (HR 3.633, 95% CI 1.681-7.848, p = 0.001), regardless of the clinical phenotype. Furthermore, we found a significant influence of a general DS on survival (HR: 2.62, 95% CI 1.335-5.160, p = 0.005).
EMG assessment could be of value not just for ALS diagnosis but also for its intrinsic prognostic value.
EMG could provide additional information about the rate of progression of ALS as early as the diagnosis is made.
验证肌电图（EMG）在诊断时发现去神经活动是否对肌萎缩侧索硬化症（ALS）具有预后价值。
我们回顾性研究了 2009 年 1 月至 2017 年 1 月期间所有出院诊断为 ALS 的患者。92 名患者符合纳入标准。我们主要验证了三个预后指标：对所有 EMG 检查进行了复查，并计算了去神经评分（DS）。对 DS 与临床里程碑的相关性进行了分析，调整了病程、年龄、性别和临床表型。
我们发现球部 DS 与 NIV/气管切开时间（HR：3.34，95%CI：1.49-7.48，p=0.002）和生存时间（HR 3.633，95%CI 1.681-7.848，p=0.001）显著相关，与临床表型无关。此外，我们发现总 DS 对生存有显著影响（HR：2.62，95%CI 1.335-5.160，p=0.005）。
EMG 评估不仅对 ALS 的诊断有价值，而且对其内在的预后价值也有价值。
EMG 可以在诊断时提供有关 ALS 进展速度的额外信息。
