The end of September saw the World Frontotemporal Dementia Awareness Week, as important as any awareness week. For those who are not familiar, Frontotemporal Dementia (FTD) is recognised as one of the most common young onset dementias, usually characterised by behaviour change, language problems and difficulties planning or organising. It is very important to note that there is an overlap between FTD and ALS, making it particularly relevant to the readers of this blog. Research has shown that up to 15% of people with ALS can develop FTD (also known as ALS-FTD or MND-FTD), which is most often characterised by changes in behaviour. Additionally, up to 50% of people with ALS can have problems in behaviour and thinking ability, similar but milder to those observed in FTD. These behaviour changes and problems with thinking abilities can be challenging for the person living with ALS and/or FTD, as well as their families.
With this week of international FTD awareness came the largest national meeting specific to this disease, hosted in London. While this meeting was primarily for practitioners and researchers, it was also open to and well attended by the public, which was highlighted by the high quality of questions and discussions during the meeting. While overall the meeting was focused on FTD, there were permeating and consistent references towards ALS (due to the degree of overlap it can have with FTD). As I was fortunate enough to attend this meeting and as a researcher in the field of ALS/MND (as well as dementia), I am keen to report and reflect on relevant research that might affect lives of those living with ALS and their families. Amidst the high quality presentations, there were two that resonated specifically in relation to ALS.
The meeting opened with Jennifer Saxon (University of Manchester) masterfully presenting her group’s findings relating to ALS-FTD. Firstly, they examined the most common problems associated with FTD that occur in ALS-FTD using retrospective research looking at historic cases. They showed that while cases of ALS-FTD mostly displayed changes in behaviour (such as apathy or lack of inhibition), there were also subgroups of people who showed a mixture of behaviour change with difficulties in language abilities as a part of their ALS-FTD syndrome. Following on from these language changes, another study presented in this series went on to explore more explicitly the profile of ALS-FTD compared with those with only FTD, once more through retrospective examination of demographics, behavioural features, language and thinking abilities. Most notably, problems in grammatical formation and comprehension of sentences (language difficulties) were most commonly observed in ALS-FTD and behaviour changes (such as social disinhibition, inertia, a loss of sympathy and empathy, repetitive behaviours and dietary changes) were most commonly observed in FTD. This emphasised the importance of the language difficulties that might occur in ALS-FTD. Jennifer finished by briefly mentioning a prospective study that comprehensively examines the ALS-FTD profile in terms of behaviour, genetics, thinking ability, language and other characteristics, so as to attempt to systematically understand what might make this combination of ALS-FTD so distinct. This will make a valuable contribution to our knowledge of these overlapping diseases; so watch this space for results of this research, which is likely to be presented at future MND/ALS meetings.
Additionally, an individual well known within the MND/ALS research community, Prof. Ammar Al-Chalabi (King’s College London) presented some thought provoking work from his group on progression of disease and the usefulness of staging to reflect progression of disease. He candidly opened by explaining the difficulties of understanding disease progression (such as it being a snapshot in time of something that continually progresses as time passes) and outlined how important it is to capture milestones in the disease through stages.
With several different disease staging methods in ALS, Prof. Al-Chalabi recommended the use of the King’s Staging System, associated with clinical progression (i.e. pathological spread of the disease) and which has also been shown to associate with functional progression (i.e. abilities and function at home). As a practical application, these can be used for timing of treatments, interventions and also serve as an end point to clinical trials. Already the value of staging has many potential benefits, and it was furthermore emphasized by Prof. Al-Chalabi that combining different methods, such as the King’s Staging and the Milano-Torino (MiToS) staging systems, would be even more integrative and helpful. However, the main focus of his talk was to point out that while staging captures many elements of the disease, currently they do not take into account people’s thinking abilities and behaviour in each stage of the disease, therefore possibly overlooking those who may have difficulties in behaviour and thinking abilities in ALS and, also relevantly, those with ALS-FTD.
Prof. Al-Chalabi finished by opening a discussion to the floor of how these thinking and behavioural problems can be used within staging, which was well received by the crowd full of people experienced within FTD. There were discussions of whether thinking and behavioural problems in certain stages should be paid more attention to, whether every stage should be described by the presence of thinking and behavioural problems or if only when there is associated FTD with ALS should thinking and behavioural problems be considered. All had merits and stimulated debate, with a resounding agreement from the audience that changes in thinking and behaviour should be included throughout the staging of disease, in one way or another. From my own standpoint, thinking, behaviour and, when present, dementia is of utmost significance and can interact with stage of disease. Furthermore, with robust methods of quantifying thinking and behaviour in ALS, it is indeed possible to explore this within each stage of disease. Finally, thinking and behaviour change can impact quality of life, adherence to treatment and also ability to make decisions, which could result in difficulties in planning for the future, decision-making and lifestyle for the people with MND/ALS and their families.