Objective

The Motor Neuron Disease Behavioural Scale (MiND-B) is a clinically validated tool that was developed to detect behavioural dysfunction in patients with amyotrophic lateral sclerosis (ALS). The current study aimed to evaluate behavioural impairment using MiND-B, as well as cognitive dysfunction in ALS patients, and to determine their prognostic implications.

Method

Patients with a clinical diagnosis of ALS were prospectively recruited from a specialised multidisciplinary ALS clinic. Patients underwent behavioural assessment with the Motor Neuron Disease Behavioural Scale (MiND-B) and cognitive evaluation using the Addenbrooke’s Cognitive Examination (ACE). Primary outcome measure was selected as survival time, defined by time from assessment to time of death or censor date. Univariate assessment of survival effect was carried out using Kaplan-Meier survival analysis followed by cox regression analysis to assess the effect of MiND-B and ACE scores on survival time.

Results

A total of 134 patients were included in the study. MiND-B testing determined that 59% were classified as having behavioural dysfunction, with deficits associated with a significantly shorter survival time (HR 2.53, p = 0.003, 95% CI 1.3–4.6). Furthermore, regression analysis demonstrated that for every 1-point reduction in the MiND-B score, risk of death increased by 3%. ACE testing established that 33% of the cohort had evidence of cognitive dysfunction. Patients with cognitive dysfunction on ACE testing had a significantly shorter survival time than patients without cognitive impairment (HR 2.0, p = 0.042, 95% CI 1.04–3.3).

Conclusion

The presence of behavioural and cognitive impairments in ALS patients was associated with poor survival. The MiND-B and ACE inventories are simple and efficient clinical tools that can be administered in the multidisciplinary ALS clinic, that aid in the prognostication of this patient population.

中文翻译：

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行为改变预示着肌萎缩性侧索硬化症的生存较差

客观的

运动神经元疾病行为量表（MiND-B）是经过临床验证的工具，旨在检测肌萎缩性侧索硬化症（ALS）患者的行为障碍。当前的研究旨在评估使用MiND-B的行为障碍以及ALS患者的认知功能障碍，并确定其预后意义。

方法

前瞻性地从专门的多学科ALS诊所招募具有ALS临床诊断的患者。使用运动神经元疾病行为量表（MiND-B）对患者进行行为评估，并使用阿登布鲁克认知考试（ACE）对患者进行认知评估。选择主要结局指标作为生存时间，该生存时间由从评估到死亡或检查日期的时间定义。使用Kaplan-Meier生存分析，然后进行Cox回归分析，评估MiND-B和ACE分数对生存时间的影响，对生存效果进行单变量评估。

结果

共有134名患者被纳入研究。MiND-B测试确定59％被归类为行为功能障碍，其缺陷与生存时间明显缩短有关（HR 2.53，p = 0.003，95％CI 1.3-4.6）。此外，回归分析表明，MiND-B得分每降低1分，死亡风险就会增加3％。ACE测试证实，该队列中有33％的人有认知功能障碍的证据。与没有认知障碍的患者相比，接受ACE检测的认知功能障碍的患者的生存时间明显短（HR 2.0，p = 0.042，95％CI 1.04–3.3）。

结论

ALS患者的行为和认知障碍的存在与生存不良有关。MiND-B和ACE清单是可以在多学科ALS诊所中进行管理的简单有效的临床工具，可帮助对该患者群进行预后。