Cognitive and behavioral impairment in amyotrophic lateral sclerosis (ALS) is now well recognized
that there is clinical, pathological and genetic overlap between ALS and frontotemporal dementia
(FTD). Although the cognitive and behavioral status of most patients is not yet fully recognized, roughly
up to half of patients with ALS develop cognitive and behavioral impairments and a smaller subgroup
of ALS patients, who impaired more severely in cognitive function and behavior, meet the criteria for
the FTD (ALS-FTD). Deficits are characterized by executive impairments, extending to changes in
language and social cognition, then implying a clinical spectrum linking ALS and FTD. Understanding
of cognitive and behavioral impairment in ALS will improve clinical management for patients and their
carers and provide valuable clues to encompass the heterogeneity of cognitive and behavioral
impairments in ALS and other neurodegenerative diseases.




KEYWORDS
Amyotrophic lateral
sclerosis,
Frontotemporal dementia,
Cognition,
Behavior