Achi Eugene Y, Rudnicki Stacy A
Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA.
Neurol Res Int. 2012;2012:806306. doi: 10.1155/2012/806306. Epub 2012 Aug 7.
Though once believed to be a disease that was limited to the motor system, it is now apparent that amyotrophic lateral sclerosis (ALS) may be associated with cognitive changes in some patients. Changes are consistent with frontotemporal dysfunction, and may range from mild abnormalities only recognized with formal neuropsychological testing, to profound frontotemporal dementia (FTD). Executive function, behavior, and language are the most likely areas to be involved. Screening helpful in detecting abnormalities includes verbal or categorical fluency, behavioral inventories filled out by the caregiver, and evaluation for the presence of depression and pseudobulbar affect. Patients with cognitive dysfunction have shortened survival and may be less compliant with recommendations regarding use of feeding tubes and noninvasive ventilation. Evolving knowledge of genetic and pathological links between ALS and FTD has allowed us to better understand the overlapping spectrum of ALS and FTD.
尽管肌萎缩侧索硬化症（ALS）曾被认为是一种仅限于运动系统的疾病，但现在很明显，在一些患者中，ALS可能与认知变化有关。这些变化与额颞叶功能障碍一致，范围可能从只有通过正式神经心理学测试才能识别的轻度异常，到严重的额颞叶痴呆（FTD）。执行功能、行为和语言是最可能受影响的领域。有助于检测异常的筛查包括言语或分类流畅性、护理人员填写的行为量表，以及对抑郁症和假性延髓情绪的评估。认知功能障碍患者的生存期缩短，并且可能不太愿意遵守关于使用喂食管和无创通气的建议。对ALS和FTD之间遗传和病理联系的不断了解，使我们能够更好地理解ALS和FTD的重叠范围。
