Sign in to CMS Desk.

Cognition

Fronto-temporal cognitive changes have been associated with MND. Fronto-temporal dementia is prominent in 5-10% of cases. Recent neuropsychological studies suggest that approximately two thirds of people with motor neurone disease may suffer from mild changes in cognitive skills and processes and/or behavioural change. (MND Australia 2014).

Strong and others 2009

There is considerable evidence supporting the existence of cognitive and behavioural dysfunction in ALS, including a spectrum of frontotemporal syndromes and more classically defined dementias. These include:
  • ALS in association with cognitive impairment (ALSci)
  • ALS with behavioural impairment (ALSbi)
  • ALS with a concurrent dementia that meets the criteria for a FTD (ALS-FTD). The core aspect of this diagnostic categorization relates to the presence or absence of a frontotemporal lobar degeneration. ALS-FTD can manifest in three clinically recognized subtypes.
    • The most common (behavioural variant FTD, bvFTD) is a progressive behavioural syndrome marked by insidious onset, altered social conduct, impaired regulation of interpersonal conduct, emotional blunting and loss of insight.
    • In addition, both a progressive non-fluent aphasia (PNFA – characterized by progressive non-fluent spontaneous speech with agrammatism, paraphasias or anomia) and semantic dementia (SD, characterized by fluent speech with impaired understanding of word meaning and/or object identity) are considered to be within the FTD spectrum.

Miller and others 2009b

What is the prevalence and natural history of cognitive and behavioral impairment in ALS?
  • Estimates of cognitive impairment range from 10% (Class III) to 75% (Class III). A population-based sample produced an estimate of 28% (Class II). The prevalence of impairment meeting criteria for dementia ranged from 15% (Class III) to 41% (Class II). Behavioral impairment (irritability and social disinhibition) was identified in 39% (Class III).
  • Three studies (Class III) documented mild cognitive decline over 6 months, while others (Class III) found no change over 12 months. It is not known whether patients can progress from ALSci or ALSbi to ALS-FTD. However, 15% of patients presenting with FTD later develop motor neuron degeneration (Class III).
  • Conclusions
    • A significant proportion of patients with ALS demonstrate cognitive impairment and some have dementia (2 Class II, multiple Class III studies).
  • Neither behavioral impairment in ALS nor the natural progression of cognitive or behavioral impairments has been adequately studied.
  • Recommendation
    • Screening tests of executive function may be considered to detect cognitive impairment in patients with ALS prior to confirmation with formal neuropsychological evaluation (Level C).

 

Referral Pathways

Click on a state or territory.

Or to search using additional criteria visit Referral Pathways.


Disclaimer Copyright MNDcare

Funded by the Australian Government Department of Health and Ageing


MND Australia would like to advise Aboriginal and Torres Strait Islander users that this website may contain images or names of deceased persons.