Alcaz S, Apostolski S
Srp Arh Celok Lek. 1995 Sep-Oct;123(9-10):266-70.
We present the facts on kind and frequency of disorder of high cortical functions in amyotrophic lateral sclerosis. It was considered for a long time that this disease affects selectively motor neurons. The signs of degeneration found in other structures and associated signs of other neurological disorders brought the diagnosis of amyotrophic lateral sclerosis under suspicion. However, the specific type of degenerative dementia is nowadays more frequently discovered in patients with amyotrophic lateral sclerosis. The nosological status of motor neuron disease with dementia is still controversial. There is a possibility of a new clinical entity or a variant of motor neuron disease. Taking into account the fact that pathological changes of motor neurons and the natural history of amyotrophic lateral sclerosis and amyotrophic lateral sclerosis with dementia are not essentially different, as well as the fact that frontal dysfunction is detected in non-demented patients with amyotrophic lateral sclerosis, the authors consider that we are facing a variant of well-known disease of motor neuron.
我们阐述了肌萎缩侧索硬化症中高级皮质功能障碍的类型及发生频率的相关事实。长期以来人们认为这种疾病只选择性地影响运动神经元。在其他结构中发现的变性迹象以及其他神经系统疾病的相关体征使肌萎缩侧索硬化症的诊断受到质疑。然而，如今在肌萎缩侧索硬化症患者中更频繁地发现了特定类型的变性性痴呆。伴有痴呆的运动神经元病的疾病分类地位仍存在争议。存在一种新的临床实体或运动神经元病变体的可能性。考虑到运动神经元的病理变化以及肌萎缩侧索硬化症和伴有痴呆的肌萎缩侧索硬化症的自然病史并无本质区别，以及在无痴呆的肌萎缩侧索硬化症患者中检测到额叶功能障碍这一事实作者认为我们面对的是一种已知运动神经元疾病的变体。
