Résumé
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by motor deficits. In recent years, cognitive impairment in ALS has been widely concerned, and the concept of ALS-frontotemporal dementia spectrum has been commonly recognized and well-established. With the development of neuropsychology, neuroimaging, neurophysiology, neuropathology and neurogenetics, advances have been made in the diagnosis and pathophysiological mechanism of cognitive impairment in ALS. The most recent advances of cognitive impairment in ALS were reviewed.
Résumé
Amyotrophic lateral sclerosis (ALS) is a rapid progressive neurodegenerative disease, characterized by the degeneration of both upper and lower motor neurons. The progressive motor unit loss due to lower motor neuron damage is one of the most important pathological process in ALS. However, there are lack of specific biomarkers for early diagnosis and progression monitoring of ALS. The relevant electrophysiologic techniques which quantify the number of motor unit have been developed rapidly in recent years. Among them, the motor unit number estimation (MUNE) and the motor unit number index (MUNIX) have been widely applied to quantify motor unit number loss in ALS. The most recent advances of MUNE and MUNIX in ALS were reviewed.