Translational and Clinical Physiology Unit, Instituto de Medicina Molecular-Faculty of Medicine, University of Lisbon, Portugal.
Clin Neurophysiol. 2012 Dec;123(12):2454-9. doi: 10.1016/j.clinph.2012.05.012. Epub 2012 Jul 3.
Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disease associated with short survival due to respiratory failure. We aimed to test the predictive value of the phrenic nerve motor response for survival, in a large population of ALS patients.
We included 254 ALS patients followed in our tertiary centre from 1997 to 2006, in whom phrenic nerve stimulation was performed according to the study inclusion and exclusion criteria. ALS was spinal onset in 175 and bulbar onset in 79 patients. The following features were recorded at entry: gender, age at presentation, onset region, diagnostic delay, forced vital capacity (FVC), ALS functional rating scale (ALS-FRS) including the respiratory subscore of the reviewed ALS-FRS and mean amplitude of motor responses by phrenic nerve stimulation (PhrenAmpl).
Survival analysis was evaluated by Kaplan-Meier log-rank test and multivariate Cox proportional hazards. Independent factors negatively affecting survival were bulbar onset, short diagnostic delay, FVC and small PhrenAmpl for the total population. Small PhrenAmpl and short diagnostic delay were also independent factors for both spinal and bulbar-onset patients; age at onset and FVC were also independent predictors in bulbar-onset patients.
Phrenic nerve stimulation is a non-volitional test that can be performed quickly in most patients; it is a powerful predictor of survival in ALS.
Phrenic nerve stimulation should be considered as an additional test for respiratory assessment in ALS.
肌萎缩侧索硬化症（ALS）是一种严重的神经退行性疾病，由于呼吸衰竭导致生存时间短。我们旨在测试膈神经运动反应对生存的预测价值，在一个大型 ALS 患者人群中。
我们纳入了 1997 年至 2006 年在我们的三级中心随访的 254 名 ALS 患者，根据研究纳入和排除标准对他们进行了膈神经刺激。ALS 脊髓起始 175 例，延髓起始 79 例。入组时记录以下特征：性别、发病年龄、起始部位、诊断延迟、用力肺活量（FVC）、肌萎缩侧索硬化功能评定量表（ALS-FRS），包括修订后的 ALS-FRS 呼吸亚评分和膈神经刺激的运动反应平均幅度（PhrenAmpl）。
通过 Kaplan-Meier 对数秩检验和多变量 Cox 比例风险进行生存分析。对总人群的生存有负面影响的独立因素为延髓起始、诊断延迟短、FVC 和 PhrenAmpl 小。对于脊髓和延髓起始患者，小 PhrenAmpl 和短诊断延迟也是独立因素；对于延髓起始患者，发病年龄和 FVC 也是独立的预测因素。
膈神经刺激是一种非自主测试，大多数患者都可以快速进行；它是 ALS 生存的有力预测因素。
膈神经刺激应被视为 ALS 呼吸评估的附加测试。
