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0:00
Welcome to the talk
on the importance
of nutrition and weight
in amyotrophic lateral
sclerosis motor neuron disease.
I have a PhD and
working as an Inserm
research director
in Strasbourg, France.
0:17
Why do we say that weight
loss is important in ALS?
Because ALS has actually
a component that is
related to metabolism.
Weight loss in ALS is
caused by multiple factors,
but two of these are actually
the most important ones.
One is decreased food intake,
which is particularly
related to dysphasia
that occurs during
disease progression
and the other one
is increased energy expenditure-
both of them might be
related to altered
hypothalamic signaling.
I hope that in this talk
I will convince you that the
hypothalamus is actually
instrumental in weight loss in
ALS patients and that
dietary intervention and
in particular high caloric,
high-fat diet might
be important in terms of
therapeutic counseling.
1:14
Another component of ALS is
related to energy metabolism
and is actually systemic.
There are pathological
abnormalities in ALS tissues
beyond the classical origins of
the CNS that are involved in ALS.
For instance, the
hypothalamus is atrophied,
there are TDP-43 aggregates,
especially in the lateral
hypothalamic area,
and there are impairments of
various hypothalamic signaling,
including melanocortin.
In the pancreas,
there have been reports of
impaired insulin secretion.
In the adipose tissue,
the tissue has been
atrophied and is abnormally
distributed as compared
to healthy controls.
Skeletal muscle shows
increased lipid usage,
impaired glucose usage, and
mitochondrial dysfunction.
