Vim & Vigor - Summer 2017 - University of Virginia - 54

WHAT GOES WRONG
IN THE BRAIN

SUMM ER 2 0 1 7

Finding Ways to Manage

For Buzz Brown, FTD has impaired his
mental faculties but has had a greater
impact on his behavior. "There was a
point," his wife shares, "that we had to
take away anything that could be used
to hurt himself or someone. We had to
get rid of all the knives and scissors in
the house."
Three years ago, the day came when
Linda knew she could no longer keep her
husband safe at home. "I had Buzz and
my grandchildren in the car. He thought
the other cars were too close and they
were going to get us. He grabbed the
wheel of the car," she says. No one was

PHOTOS BY GETTY IMAGES

It might sound like science fiction. But it's real and vitally important for any
family facing frontotemporal dementia (FTD): In a UVA Health System lab,
Erin Foff, MD, PhD, and her research team are creating miniature 3-D brain
models. They build them with stem cells taken from patients who have FTD.
The researchers use these models to test in a dish potential therapeutics
to try to stop the disease's toll on the brain. "This type of basic science is
happening all over the world," Foff says.
It's the essential groundwork needed before new therapies can be made
available. "There are clinical trials coming down the road for FTD," Foff says.
"For at least a subset of FTD patients with known genetic mutations, we are
hopefully moving fast enough-and there's enough financial investment in this
research-so these families can be encouraged by what's coming, especially if
they have children who may have inherited the same mutation."
There is still much to learn about FTD, but researchers do know that "not
every patient with FTD has the same thing happening with their brain," Foff
says. In the last several years, researchers have found different gene mutations to help explain what's going on at the cellular level. "We're learning more
about what's causing this disease, but because it's not one disease, developing
a therapeutic is very challenging," she adds.
"The way we're headed," Foff continues, "is using noninvasive imaging or
blood biomarkers to very carefully characterize each patient to understand
as much as we can about what's going on at the cellular level. We are taking
giants steps in that direction, but we are not quite there yet."

of a progressive brain disease that will
slowly steal away a person's mental faculties. Although all dementias are difficult to deal with, FTD can be even more
daunting. One type, known as Pick's
disease, changes the very essence of a
person as it shrinks the brain's frontal
lobe, the control center for judgment
and personality.
Someone with Pick's may become
withdrawn, apathetic and unable to
sense the emotions of other people.
"Their spouse could be crying and they
laugh because they no longer know what
that means," Foff says. She has seen others with this disease say things no one
would ever say out loud. "Very often,"
she adds, "they are fired from their jobs
for behaving inappropriately. Their marriage falls apart for having affairs. Or
they gamble away their life savings." All
of this usually happens before anyone
realizes they have a neurodegenerative
disease and often at the prime of their
life. Last year, one of the founders of the
'80s band Beastie Boys died from FTD.
He was 52.
A much different form of FTD starts
out by affecting language. "A person
first has difficulty producing words
or produces nonsensical words," Foff
explains. "Other cognitive problems
typically show up years later."

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