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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.
