Trojsi Francesca, Santangelo Gabriella, Caiazzo Giuseppina, Siciliano Mattia, Ferrantino Teresa, Piccirillo Giovanni, Femiano Cinzia, Cristillo Viviana, Monsurrò Maria Rosaria, Esposito Fabrizio, Tedeschi Gioacchino
a Department of Medical, Surgical, Neurological, Metabolic and Aging Sciences , Second University of Naples , Naples .
b Department of Psychology , Second University of Naples , Caserta , and.
Amyotroph Lateral Scler Frontotemporal Degener. 2016;17(3-4):228-35. doi: 10.3109/21678421.2016.1143513. Epub 2016 Feb 24.
Emerging evidence shows that cognitive deficits associated with frontal lobe dysfunction occur from early stages of amyotrophic lateral sclerosis (ALS). We aimed to assess neuropsychological functioning at different stages of ALS to further delineate the occurrence of cognitive impairment alongside the trajectory of ALS as defined by standard assessment procedures. We investigated several cognitive domains in 74 ALS patients classified into four different clinical stages of disease, according to a recently validated staging system for ALS (known as 'King's' system), and evaluated and compared the corresponding cognitive profiles. We found that data derived from global cognitive assessment and several executive (i.e. Frontal Assessment Battery and Trail Making Test B-A) and long-term memory (i.e. memory prose) tests were significantly different among the subsets of ALS patients, showing poorer performances with increasing clinical disability. In conclusion, our preliminary results support the notion that mainly frontotemporal abilities may be impaired during the ALS course and suggest that neuropsychological information could supplement the current clinical staging of patients. However, ALS-specific multi-domain screening instruments, which allow to correct neuropsychological scores for physical disability, should be validated in larger populations worldwide and routinely introduced in clinical practice.
新出现的证据表明，与额叶功能障碍相关的认知缺陷在肌萎缩侧索硬化症（ALS）的早期阶段就已出现。我们旨在评估ALS不同阶段的神经心理功能，以进一步明确认知障碍的发生情况，以及标准评估程序所定义的ALS病程。我们根据最近验证的ALS分期系统（称为“国王”系统），对74例ALS患者进行了分类，这些患者分为四个不同的疾病临床阶段，并对相应的认知特征进行了评估和比较。我们发现，来自整体认知评估以及多项执行功能（即额叶评估量表和连线测验B-A）和长期记忆（即记忆散文）测试的数据在ALS患者亚组之间存在显著差异，随着临床残疾程度的增加，表现出更差的成绩。总之，我们的初步结果支持这样一种观点，即在ALS病程中主要是额颞叶能力可能受损，并表明神经心理信息可以补充目前患者的临床分期。然而，能够针对身体残疾校正神经心理评分的ALS特异性多领域筛查工具，应在全球更大规模的人群中进行验证，并常规引入临床实践。
