ALS Center, Department of Neurology, Azienda Ospedaliera Universitaria Maggiore della Carità, Novara, Italy.
3rd Neurology Unit and Motor Neuron Diseases Centre, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 Aug;21(5-6):373-379. doi: 10.1080/21678421.2020.1771732. Epub 2020 Jun 2.
: 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.: 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.: 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.: 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.
认知障碍，从额颞叶痴呆（FTD）到更轻微的执行功能障碍或行为功能障碍，在诊断为肌萎缩侧索硬化症（ALS）的患者中 30-50%可被检测到。这种情况极大地影响了预后，并可能影响到关于生命末期选择的决策过程。我们研究的目的是检查在从诊断到 6 个月随访（IQR5.5-9.0 个月）的时间内，大量 ALS 患者的认知和行为障碍的变化，并检查认知障碍的进展在多大程度上影响生存时间和疾病进展率。
我们招募了根据肌萎缩侧索硬化症和额颞叶痴呆谱障碍修订标准分类的 146 名 ALS 患者。在多学科环境中，我们在两次后续就诊中检查了临床特征，包括 ALSFRS-r 评分、FVC%和 BMI，以及认知状态，包括广泛的神经心理学评估。
在第二次检查中，三分之一的患者出现认知障碍恶化，即 88%的 ALSbi、27%的 ALSci、40%的 ALScbi，有趣的是，还有 24%的认知正常 ALS 患者出现了显著的认知功能障碍。我们发现，那些改变了认知状态的患者在 t1 时的 ALSFRS-r 评分较低，并且与那些没有改变的患者相比，生存时间较短，无论认知障碍的类型如何。
我们展示了 ALS 患者的认知障碍不仅可以在诊断时存在，而且可以在疾病过程中表现出来，并影响运动缺陷的进展和预后。
