The biggest ALS European meeting was held this year in Dublin, Ireland. Due to unforeseen circumstances, the venue (i.e. country and city) had to be changed last minute from Milan, Italy. Nevertheless, Dublin as a city was riveting and impressive, which was further augmented by the electric atmosphere created by the same-sex marriage referendum that took place during the dates of the ENCALS meeting.
The Cognitive session will be my focus here and Prof Sharon Abrahams and Dr Marwa Elamin chaired it. The session opened with a quick introduction and an important highlight that this marks the very first Cognitive session ever held at ENCALS. While the session was mostly about Cognition in ALS (being true to its name), it was riddled with behavioural aspects of functioning as well.
Dr Marwa Elamin, from Trinity College Dublin, Ireland, begun with the session. Her talk focused on behavioural change in ALS patients without dementia. This collaborative and inter-centre study between Ireland and Scotland utilised the most effective screen for cognition and behaviour. In the presentation, Dr Elamin took a quite a patient and caregiver centred portrayal of results, giving specific and strong examples of behaviour and cognition in ALS. This engaged the crowd and helped the audience relate to the findings, and painted a clearer picture the behaviour change that can possibly occur in ALS. My own feeling was that this is an excellent way present data that relates to behaviour, as it could be qualitatively valuable. The study found that just over 40% of patients who participated in the study were reported to have behaviour change, the first and foremost of those being apathy. Other behaviour changes were also noted, showing the complex behavioural changes that may occur. The study concluded with findings that point toward further subtypes of ALS that relate to behavioural impairment and that this might be associated with performance on certain cognitive tasks, such as those relating to processing social information (social cognition) and language. This was a very intuitive finding as behavioural problems could be said to manifest in such overt parts of cognition.
As is becoming tradition, the talk that follows was my own and focused on comparing apathy profiles in ALS, Parkinson’s disease and Alzheimer’s disease. However, as I was unable to make notes on my own talk and as this time I was keen on try and avoiding any self-reporting bias, I asked my good friend, fellow colleague and great researcher Christopher Crockford to summarise my talk. So below (in quotation marks) is his observation and view of my very own talk:
“Ratko Radakovic, from the University of Edinburgh, United Kingdom, presented his study on apathy in patients with ALS. His research suggests that apathy has a number of subtypes, rather than being just one thing, which was supported by previous studies and literature. Ratko examined how apathy differs in patients with ALS, Alzheimer’s Disease and Parkinson’s Disease using the scale he developed, the Dimensional Apathy Scale. Patients with ALS had higher rates of what is called Initiation apathy, a type of apathy concerning patients’ difficulty in beginning an activity. This compares to patients with Alzheimer’s Disease who have higher rates of all forms of apathy and patients with Parkinson’s Disease who showed Initiation apathy and Executive apathy (planning, organisation and attention) when compared to healthy adults. Furthermore, there was a difference between all patient groups on Executive apathy. Finally, there was a suggestion that these findings are important for how patients might engage with medical treatments and how clinicians support patients with ALS.”
The next talk was Umberto Manera, of the University of Turin, Italy, which looked at cognitive impairment in ALS assessed with a relatively novel brain imaging technique (18F-FDG PET). With this technique, it is possible to look at the brain’s glucose metabolism and, as was presented in the talk, it is able to categorize ALS patients and healthy controls to an accuracy of 94%. With a very large number of ALS patients (N=170), they were able to further group the patient participants through comprehensive neuropsychological and behavioural assessment and compare their hypometabolism (reduced glucose metabolism) in specific brain regions. I would be at a miss if I were to summarise everything specifically, as it was a talk extremely rich with information due to the detailed imaging in this study. However, it was clear that dependent on the cognitive or behavioural status there were differences in metabolism between subdivisions of patients. It provided evidence towards particular brain area hypometabolism associations with behaviour, cognition and Frontotemporal dementia in ALS. It is the first study that postulating that it is possible to differentiate levels of cognitive or behavioural deficits in ALS by this imaging technique, which should be focused on in the future.
Last, but definitely not least, we had Tom Burke, Trinity College Dublin, Ireland, who looked to unwrap pure ALS (ALS with no cognitive impairment) and looked to discuss whether there was such a thing as pure ALS. This study took a data driven approach, or as it was playfully called the “finding the needle in the hay stack” method, to look for any possible overt, albeit important, cognitive changes in a sea of valuable ALS patient information. He was able to capitalise on the excellent facilities and Clinic based recruitment in Ireland. Through stringent and aimed inclusion and exclusion criteria in this study, clustering and careful stratification of neuropsychological data was deployed and he was able to extract key findings in relation to pure ALS. These key findings being that cognitive impairment was observed in cases where no cognitive impairment initially apparent. He talked of a longitudinal follow-up of cognition, showing a possible decline, and dispelled alternative explanations for this decline, such as various biases. With all the bases covered, the presentation ended in a great summative way, cautioning the use of the term pure ALS, also giving a honourable mention to the importance of behavioural aspects of functioning. Finally, there was an emphasis on the importance of in depth neuropsychological assessment in research and clinic practice, as to be more clear and comprehensive.
This was an excellent Cognitive session and, hopefully, marked the beginning of further focus of these behavioural and cognitive areas of research in future meetings.