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首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis
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Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis

机译：区域扩散模式预测散发性肌萎缩性侧索硬化患者的生存

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Background and purpose: Sporadic amyotrophic lateral sclerosis (sALS) is a disease with a focal clinical onset and contiguous spread. We examined patterns of disease spread following symptoms onset in sALS and whether the pattern of spread predicted survival. Methods: Review of medical records (2003-2009) at London Ontario and Buenos Aires clinic cohorts retrieved 318 patients with sporadic sALS. According to patient self-report, we determined eight spread patterns: rostro-caudal, caudo-rostral, crossed, circular, superior interposed, middle interposed, inferior interposed and isolated. The variables studied were as follows: age, gender, sALS phenotypes, time from onset to diagnosis and time and direction of the spreading to the first region. Survival from symptoms onset was analysed by Kaplan-Meier, Tarone-Ware and Cox proportional hazards methods. Results: The direction of first spread was horizontal in 33%, rostral to caudal in 32% and caudal to rostral in 21%, whereas spread to remote regions was observed in 14% of patients. Survival curves and 3- and 5-year survival rates favoured patients with an isolated and caudo-rostral pattern of spread compared to patients progressing to distant regions without involvement in the intervening region, or 'superior and inferior interposed patterns' (Tarone-Ware P=0.001, χ 2=0.002 and χ 2=0.006, respectively). Factors affecting survival were gender, time to diagnosis, flail arm phenotype and age at diagnosis. Conclusions: We have provided evidence that not all spread in ALS is contiguous and that the nature of symptom progression influences survival. Patients with sALS with 'interposed patterns' had a worse prognosis, whereas patients with caudo-rostral pattern fared better than the rest.
机译：背景与目的：散发性肌萎缩性侧索硬化症（sALS）是一种具有局灶性临床发作且连续扩散的疾病。我们检查了sALS症状发作后疾病传播的模式，以及传播的模式是否预测生存。方法：回顾伦敦安大略省和布宜诺斯艾利斯诊所队列的医疗记录（2003-2009年），发现318例散发性sALS患者。根据患者的自我报告，我们确定了八种传播方式：尾状，尾状，交叉，圆形，上置，中置，下置和隔离。研究的变量如下：年龄，性别，sALS表型，从发病到诊断的时间以及扩散到第一区域的时间和方向。通过Kaplan-Meier，Tarone-Ware和Cox比例风险方法分析症状发作后的生存率。结果：首次扩散的方向是水平的，占33％，从鼻到尾的占32％，从尾到鼻的占21％，而在14％的患者中扩散到偏远地区。生存曲线和3年和5年生存率较无进展介入区域或不涉及介入区域或“上，下介入模式”的患者更倾向于具有孤立的，呈假性延展性分布的患者（Tarone-Ware P分别为0.001，χ2 = 0.002和χ2= 0.006。影响生存的因素是性别，诊断时间，fl臂表型和诊断年龄。结论：我们提供的证据表明并非所有的ALS扩散都是连续的，并且症状进展的性质会影响生存。 sALS呈“间断型”的患者预后较差，而假鼻翼型的患者则比其他患者好。

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