[Frontal lobe dysfunction in ALS]

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.