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Cognition in ALS and the overlap with frontotemporal dementia (FTD)

Published on June 30, 2016 Reviewed on May 4, 2022   44 min

A selection of talks on Neurology

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0:00
My name is Dr. Thomas Bak and I work at the University of Edinburgh. I would like to speak about cognition in ALS and in particular about the overlap with frontotemporal dementia. Now you might start asking what is cognition in ALS, isn't it referred to as motor neuron disease which per definition should be motor. I hope that by the end of this lecture, I would have convinced you not only that cognition is a very important part for the clinical in vitro of ALS, but that it is an absolutely integral part for our understanding of the disease.
0:37
So to start with the structure of the talk, I will first speak about the history of our discovery of cognitive symptoms in ALS, addressing the question whether this is really a recent discovery or something which in fact can go back much more in time. I will then move to discuss the nature of cognitive deficits, in particular the frontal-executive dysfunction, social cognition behavior, and language. And then I would come to what I think is a central question, why is cognition affected in ALS, and finally, after hopefully convincing you that it's an important part of the disease, I want to speak a little bit about assessment of cognitive symptoms, how can we assess them, how can we really judge whether they're present or not.
1:23
So you might be surprised that the first slide I start with here is from 1893, quite long time ago and incidentally from Japan. This was until recently and arguably little known piece of work published in Journal of the Medical Society of Okayama, which would not be famous even in Japan, but I think this is very interesting. It happens to be the first description of aphasia, namely language disorder associated with brain disease in Japan. But what makes it really interesting is that this first description of aphasia was in context of motor neuron disease. So already in 19th century, physicians have noticed that at least some of the patients with ALS might have something wrong with their cognitive functions. In this case interestingly, there's a description of a disorder of speaking but also of writing and reading, affecting one part of Japanese writing, kana more than kanji, I will come to it much later where I just hope it will become relevant.

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Cognition in ALS and the overlap with frontotemporal dementia (FTD)

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