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My name is Kevin Talbot.
I am a neurologist
at the University of Oxford
in United Kingdom
and run the Oxford
Motor Neuron Disease
Care and Research Centre,
where we see patients with
amyotrophic lateral sclerosis,
and other motor
neuron disorders.
Today, I'm going to talk to you
about the clinical features
of amyotrophic lateral sclerosis
concentrating
principally on diagnosis
but also touching on natural
history and prognosis
and also on epidemiology.
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Amyotrophic lateral sclerosis
is in essence a neurotic
degenerative disease
and in that it is in common
with Parkinson's
and Alzheimer's disease,
an age related condition.
And you can see from the graph
here of the UK population
that the condition peaks
in incidence in the 70s.
The prevalence of the condition
is roughly 5 per 100,000,
which means that
in the United Kingdom
there are about 5,000 people
living with motor neuron disease
at any one time.
Throughout the world,
it's estimated there
may be several
100,000 people with ALS
although for much of the world
we don't really know
the epidemiology.
Over the lifetime
of an individual,
there's roughly a 1 in 400 risk
of developing the disease.
This is a condition
which appears to have
a focal onset
in the cortical motor
neuronal system, that's to say,
the system which controls
voluntary movement originating
at the motorcortex of the brain
and descending
to the spinal cord.
I know that there is much
that we don't know
about this process.
This is a condition that appear
to arise in a focal area
at then undergoes
contiguous spread.
Below, you'll see
the characteristic
ubiquitinated cytoplasmic
insoluble aggregates
seen at neuropathology
which form
characteristic structures
such as these skein-like inclusions
in the middle panel
and so-called Bunina bodies
in the right-hand panel.
And that you will see
staining with TDP-43.
This is a protein which normally
resides in the nucleus,
and it's the hallmark protein
of amyotrophic
lateral sclerosis.
And in the disease the protein
moves into the cytoplasm
where it forms
insoluble aggregates
and is the major
protein constituent
of the ubiquitinated inclusions.
Approximately, 95%-97%
of all patients with ALS
of this characteristic
pathology,
suggesting there are
common biological
underpinnings
for to the disease.
