Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive degeneration of upper and lower motor neurons. Prognosis is highly variable, ranging from few months to more than 30 years. 25OH vitamin D (25OH VD) blood levels have been associated with worse prognosis of ALS, but these results remain in dispute. We addressed this controversy with a prospective study and multivariate analysis to study the influence of known clinical prognostic factors of the disease and 25OH VD levels on Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) severity score (ALS-SS), as defined by the monthly rate of decline of ALSFRS-R score, to identify the factors most closely linked to the risk of worsening of the disease. Results:This prospective cohort of ALS patients recruited 127 individuals, and 105 of them met inclusion criteria. Mean age of onset was 62.2 ± 12.1 years, 32% of subjects had bulbar onset, and gender ratio was 1.44 (male/female). Mean 25OH VD level was 26.8 ± 10.8 ng/ml and was similar between males and females. Patients with 25OH VD levels <15 ng/ml had significantly higher ALS-SS at inclusion (ALS-SSi) than those with normal levels (>30 ng/ml), p = 0.011. The study of ALS-SS as calculated at the end of follow-up (ALS-SSe) was not found correlated to initial 25OH VD levels (r = -0.19; p = 0.084). Univariate analysis showed that ALS-SSe correlated with 25OH VD levels, ALS duration at inclusion, slow vital capacity (SVC) at inclusion, and SVC loss. Multivariate model showed that 25OH VD levels were independently associated with ALS-SSe: r = -0.0125, p = 0.033. Log rank test with Kaplan-Meier curves did not show significant differences of survival between the groups defined by 25OH VD levels: <15, >15 and <30, and > 30 ng/ml, p = 0.88. Conclusions: This prospective study in ALS patients confirmed previous retrospective results: ALS-SSi is significantly higher in patients with severe VD deficiency. For the first time, multivariate analysis showed that 25OH VD level was an independent prognostic factor correlated to ALS-SSe, suggesting that discrepancies between previous works could be due to confounders. It would be important that the present work be replicated in larger samples to confirm the present findings.

中文翻译：

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低25OH维生素D血液水平与肌萎缩性侧索硬化症严重程度评分较高独立相关：一项前瞻性研究的结果。

背景：肌萎缩性侧索硬化症（ALS）是一种致命的神经退行性疾病，其特征是上，下运动神经元进行性变性。预后变化很大，范围从几个月到超过30年不等。25OH维生素D（25OH VD）的血液水平与ALS的预后较差有关，但这些结果仍有争议。我们通过前瞻性研究和多变量分析解决了这一争议，以研究疾病的已知临床预后因素和25OH VD水平对修订的肌萎缩性侧索硬化功能等级量表（ALSFRS-R）严重程度评分（ALS-SS）的影响通过ALSFRS-R得分的每月下降率，确定与疾病恶化风险最紧密相关的因素。结果：这项ALS患者的前瞻性队列招募了127个人，其中有105个符合纳入标准。平均发病年龄为62.2±12.1岁，32％的受试者患有延髓性发作，性别比为1.44（男性/女性）。25OH VD的平均水平为26.8±10.8 ng / ml，男女之间相似。25OH VD水平<15 ng / ml的患者与正常水平（> 30 ng / ml）相比，纳入时的ALS-SS（ALS-SSi）明显更高，p = 0.011。未发现在随访结束时进行的ALS-SS研究（ALS-SSe）与初始25OH VD水平相关（r = -0.19; p = 0.084）。单因素分析显示ALS-SSe与25OH VD水平，包含时的ALS持续时间，包含时的慢肺活量（SVC）和SVC丢失相关。多变量模型显示25OH VD水平与ALS-SSe独立相关：r = -0.0125，p = 0.033。用Kaplan-Meier曲线进行的对数秩检验未显示25OH VD水平定义的组之间的存活率有显着差异：<15，> 15和<30，以及> 30 ng / ml，p = 0.88。结论：这项针对ALS患者的前瞻性研究证实了先前的回顾性结果：严重VD缺乏症患者的ALS-SSi明显更高。多变量分析首次显示25OH VD水平是与ALS-SSe相关的独立预后因素，这表明以前的研究之间的差异可能是由于混杂因素引起的。重要的是，将当前工作复制到更大的样本中以确认当前的发现。这项针对ALS患者的前瞻性研究证实了先前的回顾性结果：严重VD缺乏的患者中ALS-SSi明显更高。多变量分析首次显示25OH VD水平是与ALS-SSe相关的独立预后因素，这表明以前的研究之间的差异可能是由于混杂因素引起的。重要的是，将当前工作复制到更大的样本中以确认当前的发现。这项针对ALS患者的前瞻性研究证实了先前的回顾性结果：严重VD缺乏的患者中ALS-SSi明显更高。多变量分析首次显示25OH VD水平是与ALS-SSe相关的独立预后因素，这表明以前的研究之间的差异可能是由于混杂因素引起的。重要的是，将当前工作复制到更大的样本中以确认当前的发现。