This year Dublin was not only home to the annual ENCALS meeting 2015, but also to a referendum on gay marriage. One week after the meeting the buzz of the sessions is still with me. This was an excellent event and the varied scientific sessions and posters helped to build my knowledge and understanding of research into the causes and consequences of ALS/MND. Time spent in Dublin also gave me many exciting opportunities to network with colleagues across Europe. Here is an outline of the ENCALS 2015 cognition session.
The session was chaired by two inspiring ladies and world’s leading MND researchers, Prof Sharon Abrahams from University of Edinburgh and Dr Marwa Elamin from Trinity College Dublin.
Dr Marwa Elamin gave the opening presentation on predictors of behavioral change in ALS patients without dementia by giving precise and strong examples of behaviour and cognition in ALS based on a patient and carer picture. The objective of this collaborative study between Ireland and Scotland was to exploit the frequency of behavioural changes in ALS patients without dementia and to investigate the clinical and cognitive correlates of these changes based on the Edinburgh Cognitive and Behavioural Assessment Scale (ECAS). The results suggested that 36.6% of patients who participated in the study reported changes in their behaviour. The matched controls rated significantly lower (3.3%). Two most commonly reported behavioural changes were apathy and loss of sympathy. Other documented changes included perseveration and altered diet. The question was left – How common is this? Are there any predictors? The conclusion was that the ECAS provides broad and precise information regarding behaviour in ALS. Moreover, Dr Elamin suggested that the cognitive predictor for changes in behaviour in ALS patients is their performance on tasks of social cognition and language, which might indicate further subtypes of ALS patients.
This engaging presentation was followed by Ratko Radakovic from the University of Edinburgh and his apathy talk. His research suggests that nature of apathy is multidimensional and has a number of subtypes, rather than being a singular concept, which was previously supported in the literature. He presented an interesting questionnaire-based study of apathy profiles and their subtypes in patients and their carers across Amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD) and Alzheimer’s disease (AD). To examine how apathy differs across these three diseases, he used the Dimensional Apathy Scale (DAS) which he created. This measure allows the assessment of apathetic subtypes such as emotional, executive and cognitive/behavioural initiation, and is suitable for use in patient groups with motor dysfunction. The results showed that ALS patients were less apathetic than AD patients and equally as apathetic as PD patients. He also stressed the need to discuss this widely and further explore the multidimensional apathy in other neurodegenerative diseases populations in the future.
Umberto Manera from University of Turin, the next speaker of the session outlined evidence from a large study (N=170) on cognitive changes in ALS using a novel non-invasive brain imaging technique, PET with fluorine-18 which is used to monitor changes in brain metabolism over time and to determine mitochondrial functions in neurodegenerative disorders. This study looked at the possibility of identifying different patterns of cognitive changes in ALS patients using 18F-FDG-PET. Umberto and colleagues reported significant differences in metabolism across patients with definite, probable and probable laboratory-supported ALS patients with normal cognition and frontotemporal dementia. No differences were documented between patients with behaviour changes and other sub-groups. The results suggest that increase in number of astrocytes (cells that provide physical and nutritional support for neurons, transport nutrients to neurons, and digest parts of dead neurons) due to the destruction of nearby neurons might be involved in ALS-related neurodegeneration. This study is the first one assuming that it is possible to identify different levels of cognitive and behaviour changes using 18F-FDG PET.
To close the session, Tom Burke from Trinity College Dublin presented his work on cognitive deficits in Pure ALS by taking a data-driven approach and “fishing” for any hints in the Irish patient’s data. His objective was to look into neurocognitive profile of Pure ALS patients (no cognitive changes) and compare them with matched controls. The clinic-based recruitment and excellent cognitive classification and clustering of neuropsychological data allowed him to explore the differences between Pure ALS patients scoring profiles and healthy controls. Interestingly, Tom’s results suggest that ALS patients who report not to have cognitive impairments can possibly experience executive deficits. These impairments may be considered as prodromal to cognitive impairment in ALS. “Pure ALS” should be considered as a well-established term in future research.
It was an engaging start to the ENCALS 2015, and I am sure there is plenty more still to come!