Registration for a live webinar on 'Precision medicine treatment for anticancer drug resistance' is now open.
See webinar detailsWe noted you are experiencing viewing problems
-
Check with your IT department that JWPlatform, JWPlayer and Amazon AWS & CloudFront are not being blocked by your network. The relevant domains are *.jwplatform.com, *.jwpsrv.com, *.jwpcdn.com, jwpltx.com, jwpsrv.a.ssl.fastly.net, *.amazonaws.com and *.cloudfront.net. The relevant ports are 80 and 443.
-
Check the following talk links to see which ones work correctly:
Auto Mode
HTTP Progressive Download Send us your results from the above test links at access@hstalks.com and we will contact you with further advice on troubleshooting your viewing problems. -
No luck yet? More tips for troubleshooting viewing issues
-
Contact HST Support access@hstalks.com
-
Please review our troubleshooting guide for tips and advice on resolving your viewing problems.
-
For additional help, please don't hesitate to contact HST support access@hstalks.com
We hope you have enjoyed this limited-length demo
This is a limited length demo talk; you may
login or
review methods of
obtaining more access.
- Clinical Introduction
-
1. Frontotemporal dementia
- Prof. Bruce Miller
-
2. Parkinson disease
- Prof. Stanley Fahn
-
3. Atypical parkinsonian syndromes
- Dr. David Burn
-
4. Huntington's disease
- Prof. Roger Barker
- Neuroimaging
-
5. Molecular brain imaging (PET) in diseases with dementia
- Prof. Karl Herholz
- Pathology, Genetic and Molecular Aspects (1)
-
6. A molecular understanding of Alzheimer's disease
- Prof. John Hardy
-
7. Neuropathology of neurodegenerative disorders
- Prof. Jillian Kril
-
9. Ubiquitination and Alzheimer related disorders
- Prof. John Mayer
- Pathology, Genetic and Molecular Aspects (2)
-
10. The molecular biology of Huntington's disease
- Prof. David C. Rubinsztein
-
11. Metals, oxidative stress and neurodegeneration
- Prof. Ashley Bush
- Latest Developments in the Field
-
12. Animal models of tauopathy
- Prof. David Westaway
-
13. Parkinson's disease and transplants
- Prof. Roger Barker
- Archived Lectures *These may not cover the latest advances in the field
-
14. Neuropathology of neurodegenerative disorders
- Prof. Jillian Kril
-
15. Motor neurone disease: molecular basis
- Prof. Kevin Talbot
-
16. Alzheimer's disease (AD)
- Prof. John Hodges
-
17. Frontotemporal dementia syndromes
- Prof. John Hodges
-
18. Motor neurone disease: clinical aspects
- Prof. Kevin Talbot
-
19. Neuro-imaging in dementia: using MRI in routine work-up
- Prof. Philip Scheltens
-
20. Prion diseases
- Prof. Pierluigi Gambetti
-
21. Mitochondrial disorders and neurodegeneration
- Prof. Anthony Schapira
-
23. Mutations in parkinsonian syndromes
- Dr. Andrew Singleton
-
25. Frontotemporal dementia
- Prof. Bruce Miller
Printable Handouts
Navigable Slide Index
- Introduction
- Overview
- Models of degenerative dementia
- Network-based neurodegeneration
- Neuropathologic inclusions
- Frontotemporal dementia (FTD)
- Three types of frontotemporal dementia
- Pathology variants
- Chronic traumatic encephalopathy/tau
- MRI: frontoinsular atrophy
- Early FTD: speckled TDP-43 inclusions
- Three main genetic mutations
- How many familial FTD do you follow?
- ALLFTD longitudinal FTLD
- Why go to South America?
- Initiative for genetic-neural-behavioral interactions
- Disease progression in FTLD
- pGRN hyperconnectivity in 4 GRN-related networks
- Detecting brain atrophy in GRN mutation carriers
- Neurofilament (NfL) predicts state/decline
- Baseline NfL levels predict future decline in functional status and brain structure
- NfL in genetic subtypes
- Does lifestyle alter the cognitive trajectory of FTD?
- Lifestyle matters in genetic FTLD
- Lifestyle moderates genetic FTLD
- Retraining speech production fluency in nfvPPA
- Neurodegeneration: fiction and fact
- International research criteria for bvFTD
- Leaders in the neuroscience of emotion
- Medial vs. lateral orbital cortex
- Disinhibition in an FTD patient
- Crime with dementia
- Loss of empathy
- Autonomic deficits in bvFTD
- Parasympathetic activity drives behavior
- Relationship turmoil and empathy in FTD
- Brain atrophy and caregiver health
- Patient neural health and caregiver health
- Bluefield Research Consortium: GRN
- The critical role of microglia and TNFα signalling
- GRN-deficient FTD exhibits OCD-like behavior
- C1qa removal reduces synaptic pruning
- Virus-driven expression of progranulin
- Autophagy in neurodegenerative disease (1)
- Autophagy in neurodegenerative disease (2)
- Lysosomal storage features GRN deficiency
- Rare variant enrichment MFSD8 FTLD risk
- Lipid storage, homo vs. heterozygosity
- Tau Consortium
- Pure tauopathies vs. mixed tauopathy
- Tau spreads like a prion
- Functional connectivity and tau PET in PSP
- Coming next
- Psychiatric syndromes
Topics Covered
- Frontotemporal dementia (FTD)
- The three types of FTD
- Modern subtyping
- Neurofilament as a biomarker in FTD
- Non-medical interventions
- The impact of lifestyle on genetic FTD
- Burden of caregiving
- bvFTD phenotype
- Progranulin-deficient FTD
- Treatment: Bluefield and Tau Consortiums
Links
Series:
Categories:
Therapeutic Areas:
Talk Citation
Miller, B. (2022, January 31). Frontotemporal dementia [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved December 30, 2024, from https://doi.org/10.69645/DWUH1396.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Bruce Miller has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
A selection of talks on Neurology
Transcript
Please wait while the transcript is being prepared...
0:00
Good morning. My name is Bruce Miller, of A.W.
and Mary Margaret Clausen. Distinguished professor at the University of California,
San Francisco. I'm a behavioral neurologist
and I'll be talking about frontotemporal dementia today.
0:17
Overview of my talk,
I'll begin with modern subtyping of frontotemporal dementia.
Talk about molecules, pathology and genes.
Next, I will touch on some of
the non-medical interventions that we are finding have influence on the course
of degenerative disease including lifestyle and
also the effect of speech therapy in the primary progressive aphasias.
Next speak about the burden of caregiving,
which is huge in frontotemporal dementia.
I think it has implications for the care of all degenerative diseases.
Next, I'll describe the phenotype of the behavioral variant of
frontotemporal dementia with an emphasis on how this affects empathy and emotion.
And then finally, I'll talk about
two privately funded philanthropic efforts to cure frontotemporal dementia,
The Bluefield Project and The Tau Consortium.
1:16
All degenerative diseases have somethings in common.
We've learned a lot about degenerative diseases from the model systems,
their genetic forms caused by one gene or a multitude of
genes and sporadic forms for which we don't usually know the etiology.
Their cell culture and animal models allow us to take
genetic forms of frontotemporal dementia and not come in to these model systems.
In these model systems,
whether it's IPSC derived neurons, flies or mice,
we can see the progression of the disease from preclinical
to early symptomatic when they're subtle symptomatic changes.
And finally to a full blown dementia phase, which ultimately lethal.
The theme for all of this,
which was elucidated by Stan Prusiner who won the Nobel Prize for this, is that we see
abnormal protein aggregation as the cause for
the pre-clinical early asymptomatic and symptomatic phases.
And these bad proteins,
misfolded proteins spread from one cell to the next.
The spread of these diseases is evident not only in the model systems,