Frontotemporal dementia

Published on January 31, 2022   49 min

A selection of talks on Clinical Practice

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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.
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.
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,