Department of Neurology, Nagoya University Graduate School of Medicine, , Nagoya, Japan.
J Neurol Neurosurg Psychiatry. 2013 Dec;84(12):1365-71. doi: 10.1136/jnnp-2013-306020. Epub 2013 Aug 9.
To clarify the emergence of muscle weakness in regions of the body that affect survival, and deterioration in activities of daily living (ADL) in amyotrophic lateral sclerosis (ALS) patients.
We conducted a multicentre-based prospective cohort study of patients with ALS. We enrolled 401 sporadic patients with ALS. Death or the introduction of invasive ventilation was defined as the primary endpoint, and the time to five clinical markers of ADL deterioration associated with bulbar paralysis or limb weakness were defined as ADL milestones. Muscle weakness was assessed in the neck flexor muscles; the bilateral abductors of the shoulders; the bilateral wrist extensor muscles; the bilateral flexor muscles of the hips; and the bilateral ankle dorsiflexion muscles. We performed Cox proportional hazards regression analyses for the primary endpoint and the five ADL milestones, adjusting for known covariate prognostic factors for ALS.
The Medical Research Council (MRC) score for the neck flexors was the most significant prognostic factor for the primary endpoint (HR 0.74, p<0.001), loss of speech (HR 0.66, p<0.001), and loss of swallowing function (HR 0.73, p<0.001), and was one of the significant prognostic factors for loss of upper limb function, difficulty turning in bed, and loss of walking ability (p=0.001, 0.002, and 0.008, respectively). The MRC score for the neck flexors was also a significant prognostic factor for covariates of the previously reported prognostic factors.
Neck weakness is an independent prognostic factor for survival and deterioration in ADL in Patients with ALS.
阐明影响生存的身体部位肌肉无力的出现以及肌萎缩侧索硬化（ALS）患者日常生活活动（ADL）的恶化。
我们进行了一项基于多中心的前瞻性队列研究，纳入了 401 例散发性 ALS 患者。死亡或引入有创通气定义为主要终点，与延髓麻痹或肢体无力相关的 5 个 ADL 恶化的临床标志物定义为 ADL 里程碑。通过颈屈肌、双侧肩外展肌、双侧腕伸肌、双侧髋关节屈肌和双侧踝关节背屈肌评估肌肉无力。我们对主要终点和 5 个 ADL 里程碑进行了 Cox 比例风险回归分析，调整了 ALS 的已知协变量预后因素。
颈部屈肌的 MRC 评分是主要终点（HR 0.74，p<0.001）、言语丧失（HR 0.66，p<0.001）和吞咽功能丧失（HR 0.73，p<0.001）的最重要的预后因素，也是上肢功能丧失、床上翻身困难和行走能力丧失的显著预后因素之一（p=0.001、0.002 和 0.008）。颈部屈肌的 MRC 评分也是先前报道的预后因素的协变量的显著预后因素。
颈部无力是 ALS 患者生存和 ADL 恶化的独立预后因素。
