Department of Neurology, Nagoya University, 65, Tsurumai, Showa-ku, Nagoya, Aichi, 466-8550, Japan.
Department of Neurology, Japanese Red Cross Nagoya Daiichi Hospital, Nagoya, Aichi, Japan.
J Neurol. 2016 Jun;263(6):1129-36. doi: 10.1007/s00415-016-8109-0. Epub 2016 Apr 15.
The clinical courses of sporadic amyotrophic lateral sclerosis (ALS) show extensive variability. Our objective was to elucidate how age of onset influences the progression of regional symptoms and functional losses in sporadic ALS. We included 648 patients with sporadic ALS from a multicenter prospective ALS cohort. We investigated the distribution of initial symptoms and analyzed the time from onset to events affecting activities of daily living (ADL) as well as the longitudinal changes in each regional functional rating score among four groups with different ages of onset. The frequencies of dysarthria and dysphagia as initial symptoms were higher in the older age groups, whereas weakness of upper limbs was the most common initial symptom in the youngest age group. The survival times and the times from onset to loss of speech and swallowing were significantly shorter in the older age group (p < 0.001), although the times from onset to loss of upper limb function were not significantly different among the age groups. According to joint modeling analysis, the bulbar score declined faster in the older age groups (<50 vs. 60-69 years: p = 0.029, <50 vs. ≥70 years: p < 0.001), whereas there was no significant correlation between the age of onset and decline in the upper limb score. Our results showed that age of onset had a significant influence on survival time and the progression of bulbar symptoms, but had no influence on upper limb function in sporadic ALS.
散发性肌萎缩侧索硬化症（ALS）的临床病程表现出广泛的可变性。我们的目的是阐明发病年龄如何影响散发性 ALS 中区域性症状和功能丧失的进展。我们纳入了来自多中心前瞻性 ALS 队列的 648 例散发性 ALS 患者。我们研究了初始症状的分布，并分析了从发病到影响日常生活活动（ADL）的事件的时间，以及四个不同发病年龄组中每个区域性功能评分的纵向变化。在年龄较大的组中，构音障碍和吞咽困难作为首发症状的频率更高，而在上肢无力的年轻组中，最常见的首发症状是上肢无力。在年龄较大的组中，生存时间和从发病到丧失言语和吞咽功能的时间明显缩短（p<0.001），尽管从发病到丧失上肢功能的时间在不同年龄组之间没有显著差异。根据联合建模分析，在年龄较大的组中（<50 岁 vs. 60-69 岁：p=0.029，<50 岁 vs. ≥70 岁：p<0.001），球部评分下降更快，而发病年龄与上肢评分的下降之间没有显著相关性。我们的研究结果表明，发病年龄对生存时间和球部症状的进展有显著影响，但对散发性 ALS 中的上肢功能没有影响。
