Case Study: Cognitive & Behavioural Changes in MND

Dr Sharon Abrahams, a clinical neuropsychologist, has worked with colleagues to develop a new screening method for examining cognitive changes in MND patients. 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. These cognitive changes can have a detrimental effect on the quality of life of patients and families.

The Edinburgh Cognitive ALS Screen (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 which can take up to 3 hours to administer, can be used in the clinic or in the home by a trained researcher. ECAS can tell whether someone living with MND has experienced changes in their thinking, language or behaviour. ECAS 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 the letter 'g'?....

Dr Abrahams found that the screen was successful in detecting cognitive impairment in MND patients. More specifically it was able to identify the 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 assessment for measuring cognitive and behavioural changes in MND. Using this information the MND care teams can ensure relevant support services are in place and appropriate treatments are considered.

Clinical Research

Our clinical research strategy is underpinned by Scotland’s exceptional data resources and excellence in e-health research. We are therefore well positioned to develop powerful longitudinal patient or disease registries that will both improve health provision as well as enable targeted patient research including clinical trials. Some of these are highlighted below:

SMART (Scottish Motor Neurone Disease, Audit, Research and Trials)

This is a national register and audit of MND patients in Scotland that will help identify disease patterns and treatment outcomes. It also acts as a platform for access to research and further clinical trials for patients.

Contact: Laura Stephenson

ECAS (Edinburgh Cognitive ALS Screen)

ECAS is a brief, 15–20 minute, assessment of a range of cognitive and behavioural changes, designed specifically for people with MND. It makes cognitive assessment faster and more accessible as it can be used in the clinic or a patient’s home. Using this tool will help appropriate adjustments to be made in the patient’s care and support. See our case study in the left sidebar for further information.

Contact: Dr Sharon Abrahams

DART (Edinburgh Cognitive Disorders Clinic: Diagnosis Audit Research and Treatment)

The aim of this register is to record clinical information from routine assessment of patients with cognitive disorders such as memory problems. This will help clinical colleagues to gain a better understanding of the features of cognitive diseases whilst providing access to relevant research trials.

Contact: Denise Cranley

Scottish MND DNA Bank

In very rare cases – about 5%, or 1 in 20 – MND is inherited and the condition is present in more than one family member. However, even in cases with no other family member affected, there are likely to be genetic factors which predispose an individual to develop MND, most likely when combined with environmental risk factors. By studying DNA we hope to increase understanding of why and how MND develops and this approach may eventually help in the development of new treatments.

Contact: Laura Stephenson

Skin Cell Biopsies

This is an early-stage study in which researchers invite patients with a degenerative disease such as MND to donate a punch biopsy skin sample. The sample is used by colleagues in the lab to produce stem cells. The stem cells enable researchers to study the differences in brain cell function between the cells from a patient and those from a healthy donor. Click here to read more about this case study (left sidebar).

Contact: Denise Cranley

Speak:Unique – The Voicebank Research Project

Speak:Unique – the Voicebank Research Project aims to create personalised synthetic voices for use in communication aids, simply and efficiently. It is a multi-disciplinary effort that combines the expertise of informaticians, speech and language therapists, MND care teams and researchers.

Find out more at www.speakunique.org