Cognition

Table of Contents

Advances in Research #

ALS has been traditionally viewed as a disease that impacts motor function, while cognition remains intact.
Research has challenged this assumption, and it is now clear that cognitive and behavioral changes do occur in some people with ALS.

Normal Cognition: although there is a significant proportion of people with ALS who experience cognitive changes, it is estimated that most (up to 60%) retain normal cognitive function.
Mild cognitive impairment: a significant proportion (up to 50 or 75%) of people with ALS experience subtle changes in executive function, most commonly with bulbar ALS. These changes can present as difficulty with attention, verbal skills, memory, and learning, as well as behavioural changes. There may be additional factors (such as medications, fatigue, and chronic hypoventilation) that contribute to mild impairment in memory and attention.
ALS and Frontotemporal Dementia (FTD): a smaller proportion (up to 15%) of people with ALS will develop a concomitant frontotemporal dementia, more commonly in familial ALS. This typically presents as profound personality changes and breakdown in social conduct, in addition to impaired executive function skills. Possible subtypes of FTD also include Primary Progressive Aphasia (PPA) and Semantic Dementia (SD), which are characterized by impairment in expressive and receptive language.

When providing SLP services, consider the following:

Review the medical history for any secondary diagnoses.
Assess or observe for receptive/expressive language and/or cognitive communication difficulties – this may impact the individual’s ability to use compensatory strategies (for speech or swallowing), as well as the ability to learn how to use AAC systems.
For speech/communication intervention, consider communication partner strategies (see handout ) and low tech communication aids.

Strong, M. J., Grace, G. M., Orange, J. B., & Leeper, H. A. (1996). Cognition, language, and speech in amyotrophic lateral sclerosis: a review. Journal of Clinical and Experimental Neuropsychology, 18(2), 291-303.
Yorkston, K., Miller, R., Strand, E., & Britton, D. (2013). Management of Speech and Swallowing in Degenerative Diseases. 3rd edn (Austin, TX: PRO-ED).