Flöel A, Lohmann H, Knecht S
Klinik und Poliklinik für Neurologie, Universitätsklinikum Münster.
Nervenarzt. 2002 Dec;73(12):1144-52. doi: 10.1007/s00115-002-1383-3.
Amyotrophic lateral sclerosis (ALS) has traditionally been assumed to be a purely motor disturbance. It is now recognized that 2-5% of patients with ALS develop frontotemporal dementia. Additionally, neuropsychological analysis and functional imaging suggest that a proportion of patients with classical ALS also have neuropsychological impairment. On postmortem examination, ALS patients classified as not demented showed atrophy of the frontotemporal cortex. Conversely, in patients with frontotemporal dementia without known motor impairment, atrophy of spinal and bulbar neurons were found on postmortem examination. It is still not known whether patients with ALS and neuropsychological impairment form a distinct subgroup or if they are part of a continuous spectrum that runs from pure motor impairment to pure neuropsychological impairment.
传统上，肌萎缩侧索硬化症（ALS）被认为是一种纯粹的运动障碍。现在人们认识到，2%至5%的ALS患者会发展为额颞叶痴呆。此外，神经心理学分析和功能成像表明，一部分典型的ALS患者也存在神经心理学损伤。尸检时，被归类为无痴呆的ALS患者显示出额颞叶皮质萎缩。相反，在无已知运动障碍的额颞叶痴呆患者中，尸检发现脊髓和延髓神经元萎缩。目前仍不清楚，伴有神经心理学损伤的ALS患者是构成一个独特的亚组，还是属于从纯运动损伤到纯神经心理学损伤的连续谱系的一部分。
