Written by Rachel Boothman and Kaye Stevens
This blog is part of the ‘Highlights from Glasgow’ collection of articles, where you can read about the content of some of the talks and posters presented at the 29th International Symposium on ALS/MND.
Exploration into cognition and frontotemporal dementia with MND/ALS continues to attract attention. At the Symposium in Glasgow 2018, we heard of several studies adding to the growing knowledge bank in this field.
A history of other mental health conditions or psychiatric disorders within the family, or for the individual, indicates a correlation with MND/FTD. There seems to be a link between this increased history and a risk of apathy. Early screening is recommended where these histories exist. This can help prepare families and enable early discussions with the person diagnosed with MND, so they can make decisions that may be important to them in the future (C41) C McHutchison.
We are all subject to apathy at times, where we lack motivation to accomplish our goals. However, where cognitive change is involved this can be more marked. Using a five minute questionnaire based on the Dimensional Apathy Scale (DAS), with patients and their care givers, research has found that up to 50% of people with MND/ALS experience apathy throughout the progression of the disease (C53) R Radakovic. Initiation apathy is most typical which makes it hard to accept new things, such as support from an external care worker. This can impact on burden for care givers and quality of life for both patient and care giver. External stimulation can help, so finding out what motivates the person enables activity and therapy to be customised to individual need. This is consistent with sessions at the Allied Professionals Forum which highlighted the use of music therapy and art therapy (Alisa Apreleva, Vivianna Faierman). Also, volunteers working with people who have cognitive change to stimulate interest (Marjolein Cleaver).
With cognitive change there is a greater burden on care givers than with physical demands of care support alone. Early screening and recognition of signs may help to prepare and improve the support given to carers, for example, metabolic changes and overeating indicate the potential for cognitive change. Also, making unexpected decisions about finance, wills or end of life planning that conflict greatly with previous views. Those with the gene C9orf72 may be more predisposed towards frontotemporal dementia and the MND/FTD combination is more likely to have a genetic cause (C64) J Hodges.
People with cognitive change are more likely to drop out of studies and trials. When looking at why this attrition occurs, factors common to cognitive or behavioural change became apparent, such as advancing disease stage, age of onset, fewer years of education and the presence of the genetic mutation C9orf72 (C65) C Crockford. There was a recommendation that cognitive assessment should take place during diagnosis, be monitored throughout the disease course and featured as standard in any further work on defining the stages of MND.
Typically, there isn’t a great deal of emphasis on memory decline in MND. However, a study has shown that clinicians should pay attention to memory function in older patients with cognitive impairment where episodic memory deficits may be a notable feature (C66) J Machts. Those intact at the start are unlikely to show deficits, whereas those already impaired at the start are more likely to develop deficits in, as yet unaffected areas.
If there is a past family or personal history of mental disorder or alcohol abuse, men with MND/FTD are more likely to develop psychosis. This psychosis should possibly be considered as a distinct behavioural subtype, which appears to be made up predominantly of males with the genetic mutation C9orf72 and FTD. Surprisingly, survival in this subtype seems to be prolonged. There needs to be prompt referral to psychiatric services and this form can be very difficult for carers to manage (C67) R Ahmed.
All presentations in this section indicate the need for early screening to prepare families, relieve care giver burden through better support and enable opportunities for decision making before impairment progresses.
Find out more about the topics discussed in Glasgow on our Periodic table of Symposium at www.mndassociation.org/symplive.