Dary-Auriol M, Ingrand P, Bonnaud V, Dumas P, Neau J P, Gil R
Service de Neurologia, CHU la Milètrie, Poitiers.
Rev Neurol (Paris). 1997 May;153(4):244-50.
Typical amyotrophic lateral sclerosis (ALS) is described as a motoneuron disease which spared cognitive functions. Recent studies reported cognitive impairement associated with classical ALS. Gallasi and al. (1985) detect subtle cognitive impairement sparing memory in a population of 22 patients affected with sporadic motoneuron disease. Iwasaki and al. (1990) finds lower scores, including memory tests. Our study evaluated 26 patients compared with 26 control subjects with neurospychological tests (rapid evaluation of cognitive function fluency, Weschler adult intelligence scale, Wisconsin cards, Rey scheme, memory tests - Luria -, trail making, visual retentional test of Benton Violon Seyll test). All the neuropsychological tests were significantly lower for the patients group. The cognitive impairement is global: memory and frontal functions were not spared and this impairement is also subtle. It may easily go undetected without tests. We cannot isolate a cortical or subcortical profile of the deterioration.
典型的肌萎缩侧索硬化症（ALS）被描述为一种不影响认知功能的运动神经元疾病。最近的研究报道了与经典ALS相关的认知障碍。加拉西等人（1985年）在22例散发性运动神经元疾病患者中检测到轻微的认知障碍，记忆功能未受影响。岩崎等人（1990年）发现包括记忆测试在内的得分较低。我们的研究通过神经心理学测试（认知功能流畅性快速评估、韦氏成人智力量表、威斯康星卡片、雷伊图表、记忆测试 - 卢里亚法、连线测验、本顿视觉保持测验）对26例患者与26例对照受试者进行了评估。患者组的所有神经心理学测试结果均显著较低。认知障碍是全面的：记忆和额叶功能也未幸免，而且这种障碍也很轻微。如果不进行测试，很容易被忽视。我们无法区分皮质或皮质下的恶化情况。
