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Printable Handouts
Navigable Slide Index
- Introduction
- ALS is an age-related system degeneration
- Corticospinal tract (UMN) degeneration in ALS
- Clinical presentation of ALS
- Limb onset ALS: typical clinical presentation
- Upper limb weakness/wasting: differential diagnosis
- Limb onset ALS: upper and lower motor neurons
- Corticobulbar onset ALS: typical presentation
- Corticobulbar onset ALS: differential diagnosis
- Clinical features of motor neuron disease (MND)
- Investigation of a suspected case of ALS
- Role of EMG
- Atypical presentation of ALS
- ALS is a clinical diagnosis
- Revised El Escorial criteria
- Low rate of misdiagnosis
- Average time from symptom onset to diagnosis
- Conveying the diagnosis
- Regional syndromes
- Lower motor neuron predominant ALS
- Isolated corticospinal tract degeneration
- ALS is a heterogeneous clinical syndrome
- Survival from symptom onset
- Survival distribution by clinical syndrome
- Descriptive (multi-axial) prognostication
- Age distribution on onset
- Life expectancy in UK
- Absolute incidence of MND
- Familial ALS
- Autosomal dominant inheritance
- Sporadic ALS vs. Familial ALS
- ALS & Frontotemporal Dementia (FTD)
- Cognitive impairment in ALS
- Emotional reflex hypersensitivity
- Summary
Topics Covered
- Onset & clinical features
- Differential diagnosis
- Investigation of a suspected case of ALS
- Clinical diagnosis
- Regional syndromes
- Survival & progression
- Age distribution & life expectancy
- Familial ALS
- Frontotemporal dementia
- Cognitive & emotional impairment in ALS
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Therapeutic Areas:
Talk Citation
Talbot, K. (2016, June 30). The clinical features of amyotrophic lateral sclerosis: diagnosis, natural history and epidemiology [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved October 12, 2024, from https://doi.org/10.69645/URVQ3371.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Kevin Talbot has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
The clinical features of amyotrophic lateral sclerosis: diagnosis, natural history and epidemiology
Published on June 30, 2016
33 min
Other Talks in the Series: ALS and Other Motor Neuron Disorders
Transcript
Please wait while the transcript is being prepared...
0:00
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.
0:24
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.
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