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Cognitive Impairment in Amyotrophic Lateral Sclerosis: A Consideration for Occupational Performance

Traditionally, amyotrophic lateral sclerosis (ALS) has been viewed as a degenerative disease, selective to the motor system. Until recently, research into cognitive impairment in ALS was confined primarily to a small proportion of patients with overt and clinically evident frontotemporal dementia (ALS/FTD). However, evidence from contemporary neuropsychological analysis and functional imaging studies, as identified in this review, indicates that a significantly higher percentage of patients with ALS presents with milder cognitive impairment in frontotemporal function. These deficits are considered progressive in nature, with increasing disability. Disturbances within the supervisory attentional system or central executive are thought to be responsible. It would appear that patients diagnosed with ALS may not be equally susceptible to developing cognitive deficits. The relationship between bulbar-onset ALS and increased cognitive decline is noteworthy. In ALS, the impact of cognitive disturbance on occupational performance must be considered in addition to physical disability.