Screening for Cognitive and Behavioural Change in MND

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sharon abrahams and princess royalMND Association-funded researcher Dr Sharon Abrahams (University of Edinburgh) has recently published an article on the Edinburgh Cognitive ALS Screen (ECAS) in the prestigious journal Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.

It is now recognised that, in up to 50% of people living with MND not only the motor system (walking, talking breathing etc) but also other areas of the brain, particularly those involved in thinking, language and behaviour are affected.

Frontotemporal Dementia

Cognitive and behavioural changes are increasingly common in MND. It is also well known that a small proportion of people living with MND display features of frontotemporal dementia.

For more information on cognitive change see our care information sheet.

Frontotemporal dementia (previously known as Pick’s disease) is caused by progressive degeneration in the brain’s frontal and temporal lobes (the areas of the brain involved in personality, language, higher order thinking such as decision making and behaviour). Frontotemporal dementia has an earlier age of onset compared to other forms of dementia (such as Alzheimer’s) and is usually diagnosed in the 50s and early 60s.

Back in 2011, an international collaboration, including three MND Association-funded researchers showed a link between MND and frontotemporal dementia. This link was due to the newly discovered C9orf72 gene found to be present in both sets of individuals with a family history (known as inherited MND and inherited frontotemporal dementia). Inherited MND is a rare form of MND (5-10% of total MND cases), that is characterised by a family history of the disease.

More information on inherited MND can be found via our website.

Edinburgh Cognitive and Behavioural ALS Screen (ECAS)

Dr Sharon Abrahams, University of Edinburgh (right) and her European collaborator Prof Orla Hardiman, Dublin, Ireland
Dr Sharon Abrahams, University of Edinburgh (right) and her European collaborator Prof Orla Hardiman, Dublin, Ireland

ECAS is a brief 15-20 minute assessment of a range of cognitive and behavioural changes, which is designed specifically for people living with MND. This screen, unlike existing measures of cognitive change, can be used in the clinic or in the home by a specialist nurse. ECAS can tell whether someone living with MND has experienced changes in their thinking, language or behaviour. The screen not only looks at changes that may occur in MND, but also assesses changes in memory, which might be found in other neurological diseases such as Alzheimer’s.An example of a question from the screen to assess executive function is: How many words can you think of beginning with a specific letter?

Dr Abrahams research published  in June on 40 ‘healthy’ controls and 48 people living with MND found that the screen was successful in detecting cognitive impairment in MND. More specifically it was able to identify specific areas of change, including language impairment as well as changes in the ability to make decisions. From these results ECAS has shown to be a quick and effective in-clinic assessment for measuring cognitive and behaviour changes in MND.

What’s next?

On completion of this research Dr Abrahams believes that clinicians will be able to have confidence in a rapid and easy-to-use method for detecting cognitive change, allowing for appropriate adjustments to be made in the care and support for people living with MND.

Dr Abrahams has also established collaborations with European researchers in the Netherlands, Ireland, Italy, Switzerland and Germany to use ECAS more widely, with local translations into other languages.

Commenting on her research, Dr Abrahams explained what it all means: “Although cognitive and behaviour changes are increasingly recognized within MND, the cognitive status of the majority of people attending clinics is unknown. The ECAS is a brief within clinic assessment we have developed with funding from the MND Association.

“It is designed for a specialist nurse to provide information on whether a person has experienced changes in cognition or behaviour in addition to the motor symptoms. This short interview will determine who has these changes, how severe they are and what type of changes the person is experiencing.

“Using this information we can then help with providing appropriate care and support to those individuals. We are delighted that it has begun to be adopted widely by centres in the UK and in Europe, and are very much thankful for the MND Association for supporting this work.”


Abrahams S, Newton J, Niven E, Foley J, Bak TH (2013). Screening for cognition and behaviour changes in ALS. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration. doi:10.3109/21678421.2013.805784 (Freely available by means of Open Access)

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