Xu Zhouwei, Alruwaili Ashwag Rafea S, Henderson Robert David, McCombe Pamela Ann
The University of Queensland, Brisbane, Centre for Clinical Research, Queensland, Australia.
The University of Queensland, Brisbane, Centre for Clinical Research, Queensland, Australia.
J Neurol Sci. 2017 May 15;376:16-23. doi: 10.1016/j.jns.2017.02.061. Epub 2017 Feb 27.
To screen for cognitive and behavioural impairment in people with amyotrophic lateral sclerosis (ALS) and controls with neuromuscular disease and to correlate these with clinical features.
108 people with ALS and 60 controls with other neuromuscular diseases were recruited and assessed with the Addenbrooke's cognitive examination-III (ACE-III), the frontal assessment battery (FAB), and the executive function component of the Edinburgh cognitive and behavioural ALS screen (ECAS). The Amyotrophic lateral sclerosis-Frontotemporal dementia questionnaire (ALS-FTD-Q) and the Motor Neuron Disease Behavioural instrument (MiND-B) were administered to the caregivers of people with ALS. The prevalence of abnormalities was determined and correlated with clinical features and survival. In 37 people with ALS, serial studies were performed.
The frequencies of cognitive impairment based on the ACE-III and FAB were 30.0% and 14.0%, in ALS and 11.7% and 3.3% in controls, respectively. Age and years of education influence the results of the ACE-III and ECAS executive function. In ALS, the frequencies of behavioural impairment based on ALS-FTD-Q and MiND-B were 32.1% and 39.4%, respectively. There is significant correlation of ALS-FTD-Q and MiND-B with the ALSFRS-R score. ALS participants with cognitive impairment measured with ACE-III had significantly shorter survival time than those without. ALS participants with behavioural impairment measured with ALS-FTD-Q had worse prognosis than those without. No significant difference was found between the first two serial cognitive tests based on ACE-III and FAB by using generalized estimating equation.
There is a greater frequency of cognitive impairment in people with ALS than in patients with other neuromuscular diseases. The cognitive and behavioural tests are potential biomarkers of the prognosis of ALS. The results of cognitive tests are stable over 6months and possibly longer.
筛查肌萎缩侧索硬化症（ALS）患者以及患有神经肌肉疾病的对照者的认知和行为障碍，并将这些障碍与临床特征相关联。
招募了108例ALS患者和60例患有其他神经肌肉疾病的对照者，使用剑桥认知评估量表第三版（ACE-III）、额叶评估量表（FAB）以及爱丁堡认知与行为ALS筛查量表（ECAS）的执行功能部分对他们进行评估。向ALS患者的照料者发放肌萎缩侧索硬化症-额颞叶痴呆问卷（ALS-FTD-Q）和运动神经元疾病行为量表（MiND-B）。确定异常的患病率，并将其与临床特征和生存率相关联。对37例ALS患者进行了系列研究。
基于ACE-III和FAB的认知障碍发生率在ALS患者中分别为30.0%和14.0%，在对照者中分别为11.7%和3.3%。年龄和受教育年限会影响ACE-III和ECAS执行功能的结果。在ALS患者中，基于ALS-FTD-Q和MiND-B的行为障碍发生率分别为32.1%和39.4%。ALS-FTD-Q和MiND-B与ALSFRS-R评分存在显著相关性。用ACE-III测量出有认知障碍的ALS参与者的生存时间明显短于无认知障碍者。用ALS-FTD-Q测量出有行为障碍的ALS参与者的预后比无行为障碍者更差。使用广义估计方程对基于ACE-III和FAB的前两次系列认知测试进行比较，未发现显著差异。
ALS患者的认知障碍发生率高于其他神经肌肉疾病患者。认知和行为测试是ALS预后的潜在生物标志物。认知测试结果在6个月甚至可能更长时间内保持稳定。
