0:00
Hello.
I am Philip Van Damme from the
University of Leuven Belgium.
It is my pleasure to contribute
to this lecture series about ALS,
and I will talk about the role
of neuroinflammation in ALS.
The title of my talk
is, "NeuroInflammation
in ALS, Cause or Consequence?"
0:20
The signs and symptoms of
amyotrophic lateral sclerosis,
or ALS are caused by the
degeneration of upper motor neurons
in the motor cortex
and lower motor neurons
in the brain stem and spinal cord.
This degeneration of motor
neurons is the hallmark of ALS,
but it can manifest
itself in various ways.
ALS is a heterogeneous disorder
with striking variability
in clinical presentation,
in the genetic causes,
and in the underlying
disease proteins.
Already at the clinical level,
ALS can come in different flavors.
The side of onset is
mostly focal width onset
in limb muscles or bulbar muscles.
The age at onset has a
wide range, from around
18 years of age to over 85 years.
The rate of disease progression
can be fast or slow.
And the extent of upper versus
lower motor neuron involvement,
and the extent of frontal
temporal involvement
varies from patient to patient.
Also, the genetic causes that can be
identified in about 10% of patients
range from missense mutations
in superoxide dismutase one,
or SOD1, tar DNA-binding protein
for T3, or TARDBP,
and fused in sarcoma, or FUS.
To repeat expansions of
a G-4, C-2 hexanucleotide
repeat expansion in
the chromozome 9 open
reading frame 72, or C9orf72 gene.
The underlying disease
protein is mostly TDP-43,
but can also be FUS, SOD1,
or dipeptide repeats in the
different genetic subtypes of ALS.
