OBJECTIVE To verify whether the finding of denervation activity on EMG at the time of diagnosis has a prognostic value in amyotrophic lateral sclerosis (ALS). METHODS 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. RESULTS 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). CONCLUSION EMG assessment could be of value not just for ALS diagnosis but also for its intrinsic prognostic value. SIGNIFICANCE EMG could provide additional information about the rate of progression of ALS as early as the diagnosis is made.

中文翻译：

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肌萎缩侧索硬化肌电图去神经支配结果与预后里程碑的相关性：来自回顾性研究的数据

目的 验证诊断时肌电图失神经活动的发现是否对肌萎缩侧索硬化症 (ALS) 具有预后价值。方法 我们回顾性研究了 2009 年 1 月至 2017 年 1 月期间诊断为 ALS 的所有出院患者。92 例患者符合纳入标准。我们主要验证了三个预后指标：回顾所有肌电图检查并计算去神经支配评分（DS）。分析了 DS 与临床里程碑的关联，调整了疾病持续时间、年龄、性别和临床表型。结果 我们发现延髓 DS 与 NIV/气管切开术时间（HR：3.34，95% CI：1.49 至 7.48，p = 0.002）和存活率（HR 3.633，95% CI 1.681-7.848，p = 0.001）之间存在显着相关性，无论临床表型如何。此外，我们发现一般 DS 对生存率有显着影响（HR：2.62，95% CI 1.335-5.160，p = 0.005）。结论 EMG 评估不仅对 ALS 诊断有价值，而且对它的内在预后价值也有价值。意义 肌电图可以在做出诊断时提供有关 ALS 进展速度的额外信息。