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- Clinical Introduction
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1. Frontotemporal dementia
- Prof. Bruce Miller
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2. Parkinson disease
- Prof. Stanley Fahn
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3. Atypical parkinsonian syndromes
- Dr. David Burn
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4. Huntington's disease
- Prof. Roger Barker
- Neuroimaging
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5. Molecular brain imaging (PET) in diseases with dementia
- Prof. Karl Herholz
- Pathology, Genetic and Molecular Aspects (1)
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6. A molecular understanding of Alzheimer's disease
- Prof. John Hardy
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7. Neuropathology of neurodegenerative disorders
- Prof. Jillian Kril
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9. Ubiquitination and Alzheimer related disorders
- Prof. John Mayer
- Pathology, Genetic and Molecular Aspects (2)
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10. The molecular biology of Huntington's disease
- Prof. David C. Rubinsztein
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11. Metals, oxidative stress and neurodegeneration
- Prof. Ashley Bush
- Latest Developments in the Field
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12. Animal models of tauopathy
- Prof. David Westaway
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13. Parkinson's disease and transplants
- Prof. Roger Barker
- Archived Lectures *These may not cover the latest advances in the field
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14. Neuropathology of neurodegenerative disorders
- Prof. Jillian Kril
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15. Motor neurone disease: molecular basis
- Prof. Kevin Talbot
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16. Alzheimer's disease (AD)
- Prof. John Hodges
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17. Frontotemporal dementia syndromes
- Prof. John Hodges
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18. Motor neurone disease: clinical aspects
- Prof. Kevin Talbot
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19. Neuro-imaging in dementia: using MRI in routine work-up
- Prof. Philip Scheltens
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20. Prion diseases
- Prof. Pierluigi Gambetti
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21. Mitochondrial disorders and neurodegeneration
- Prof. Anthony Schapira
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23. Mutations in parkinsonian syndromes
- Dr. Andrew Singleton
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25. Frontotemporal dementia
- Prof. Bruce Miller
Printable Handouts
Navigable Slide Index
- Introduction
- Background
- Pathophysiology: classical view
- Pathophysiology: new view
- What does this look like in the clinic?
- What does this look like clinically? The complete view
- How is the clinical service organised?
- Motor features
- The earliest clinical motor features
- Hand tapping
- Clinical features of established disease
- Cognitive features
- Clinical tests: Stroop and symbol digit
- Do all patients develop dementia?
- Hippocampal dysfunction and Huntington’s disease
- Psychiatric problems
- Social cognition
- Reading the Mind in the Eyes Test
- Weight loss and poor sleep
- Weight loss and poor sleep studies
- Sleep problems do occur in premanifest Huntington’s disease
- “Standard” treatments
- Other issues
- Disease modifying treatments being considered (2019)
- IONIS-HTTRx first clinical study
- Conclusions
Topics Covered
- Clinical features of Huntington’s Disease
- Basic pathogenic mechanisms and pathology
- Current drug therapies
- New disease modifying therapies
- Neural transplants
Links
Series:
Categories:
Therapeutic Areas:
Talk Citation
Barker, R. (2021, May 30). Huntington's disease [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved December 8, 2024, from https://doi.org/10.69645/IJUZ7361.Export Citation (RIS)
Publication History
Financial Disclosures
- There are no commercial/financial matters to disclose.
A selection of talks on Neuroscience
Transcript
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0:00
Today I'm going to talk about Huntington's disease.
My name's Roger Barker,
I'm a consultant neurologist at
the Addenbrooke's Hospital in Cambridge in the United Kingdom,
as well as the Professor of Clinical Neuroscience.
I look after patients with Huntington's disease, and
we also do research both within the lab and in the clinic into this condition.
In this topic, I'm going to take through a little of the background of Huntington's disease,
and then talk a little bit about certain aspects of it.
I hope this will help you to understand a bit more what this condition
entails, and some of the new developments which have happened more recently,
in terms of our understanding of both how it expresses itself in the clinic and some of the new treatments.
0:39
Huntington's disease takes its name from
George Huntington who described the condition in 1872.
It wasn't until 1983 that the genetic link to chromosome 4 was discovered,
and in 1993 the gene itself was found which codes for huntingtin protein.
It's an autosomal dominant condition, and the abnormal gene codes for an
abnormal form of the huntingtin protein (htt).
Autosomal dominant means that if you inherit one abnormal gene,
then you will definitely get the condition.
For most people with Huntington's disease
they present between the ages of 30 and 50, but it can present at any age.
There are people who present under the age of 21 who are said to
have 'juvenile Huntington's disease'.
There are people who have late-onset Huntington's disease,
the age for which is a bit variable, but most people would say
it's over the age of 60 or 70.
Certainly, my experience has been in recent years that
we're seeing more people presenting
later in life with the first manifestations of this condition.
Once it develops (or becomes clinically manifest)
it progresses over 20 years, and
most patients will sadly die of this condition rather than with it.
It's a relatively rare condition, in the United Kingdom
there are between 5,000 and 6,000 thousand cases, in comparison
there are 130,000 cases of
Parkinson's disease, and over half a million people with Alzheimer's disease.
So it's a rare condition,
it is a genetic disorder for which we know the gene and we can test patients.
It does have special prominence in the world of research around neurodegeneration.
In my part of the world in Cambridge,
there is a town up the A14 called Huntingdon.
The disease is often referred to as that by many physicians,
which is incorrect because (as I said)
it takes its name from George Huntington.
We've also moved away from calling it Huntington's 'chorea',
which is the movement disorder associated with it,
to emphasize that the disorder is much more
disseminated in terms of its clinical features as well as its pathology.
We refer to it as Huntington's disease.
As with all diseases we often try and find someone famous
who's had this condition, but we slightly struggle with Huntington's disease.
It is described in Ian McEwan's novel
'Saturday' where one of the main protagonists has this condition.
Probably the most famous version is Woody Guthrie, that most people will never have heard of.
He was a folk musician in the United States in the mid-part of the last century.
The Guthrie Foundation was set up in memory of him, that then led
to the registry of the disease foundation, and that in turn led to the discovery of the gene.
From Woody Guthrie, major research and discoveries have been
made in this condition.