Background: A cognitive impairment, ranging from frontotemporal dementia (FTD) to milder forms of dysexecutive or behavioral dysfunction, is detected in 30–50% of patients affected by amyotrophic lateral sclerosis (ALS) at diagnosis. Such condition considerably influences the prognosis, and possibly impacts on the decision-making process with regards to end-of-life choices. The aim of our study is to examine the changes of cognitive and behavioral impairment in a large population of ALS from the time of diagnosis to a 6-month follow-up (IQR 5.5–9.0 months), and to examine to what extent the progression of cognitive impairment affects survival time and rate of disease progression.

Methods: We recruited 146 ALS patients classified according to revised criteria of ALS and FTD spectrum disorder. In a multidisciplinary setting, during two subsequent visits we examined clinical features with ALSFRS-r score, FVC% and BMI, and cognitive status with an extensive neuropsychological evaluation.

Results: At second examination, one-third of patients showed a worsening of cognitive impairment, namely 88% of ALSbi, 27% of ALSci, 40% of ALScbi, and, interestingly, also 24% of cognitive normal ALS developed a significant cognitive dysfunction. We find that those who changed their cognitive status presented a lower ALSFRS-r score at t1 and a shorter survival time compared to those who did not change, regardless of the type of cognitive impairment.

Conclusion: We show how cognitive disorders in ALS patients can not only be present at diagnosis, but also manifest during disease and influence the progression of motor deficit and the prognosis.

背景：在诊断时，30-50% 的肌萎缩侧索硬化症 (ALS) 患者存在认知障碍，从额颞叶痴呆 (FTD) 到较轻微的执行障碍或行为功能障碍。这种情况显着影响预后，并可能影响有关临终选择的决策过程。我们研究的目的是检查大量 ALS 人群从诊断到 6 个月的随访（IQR 5.5-9.0 个月）认知和行为障碍的变化，并检查进展到何种程度认知障碍影响生存时间和疾病进展速度。

方法：我们招募了 146 名根据修订的 ALS 和 FTD 谱系障碍标准分类的 ALS 患者。在多学科环境中，在随后的两次访问中，我们使用 ALSFRS-r 评分、FVC% 和 BMI 以及认知状态检查了临床特征，并进行了广泛的神经心理学评估。

结果：在第二次检查中，三分之一的患者表现出认知障碍恶化，即 88% 的 ALSbi、27% 的 ALSci、40% 的 ALScbi，有趣的是，还有 24% 的认知正常 ALS 出现了明显的认知功能障碍. 我们发现，无论认知障碍的类型如何，与没有改变认知状态的人相比，改变认知状态的人在 t1 时的 ALSFRS-r 评分较低，生存时间较短。

结论：我们展示了 ALS 患者的认知障碍如何不仅在诊断时出现，而且在疾病期间表现出来并影响运动缺陷的进展和预后。