Frontotemporal dementia commonly afflicts people in the prime of their lives and gives them an entire new persona.
Every act of memory is to some degree an act of imagination. – Oliver Sacks
This article is dedicated to my mother, Nancy Wren Cheatham. You are in our sunrises and sunsets.
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On a Cape Cod summer weekend perhaps a decade ago, just like in previous years, the Cartolano family is set to perform their version of a talent show they call The Carto Show. It’s a pleasant seventy-four degrees, and the wind gusts from the water, swaying the juniper and scrub pines as the day fades. From the wooden deck stage, you can hear the seagulls cry and the roar of the surf. They most likely had a family barbecue that day, complete with ice cream and cake and coffee, though it’s difficult to recall now. Nina Cartolano’s mom, Rene, dressed in a nightgown as Lucille Ball, would perform Neal Diamond’s Sweet Caroline, crooning to the dog. It was heartfelt, funny entertainment, and the Cartolano’s treated it as such, said Nina, 32, who uses her comedy upbringing as a writer of sitcoms in New York.
I’ll Crush Your Fingers
For as long as she can remember, Nina’s mother would dress up in costumes and practice lines and even the choreography, just like a Broadway actress. The themes for 2014’s performance were all as varied as past summers, including appearances by her father, Leo, as the Pillsbury Dough Boy in a bathing suit, and the family performing a rewritten Can’t Buy Me Love. Except this year was different, as one person remained aloof from the production, sitting peacefully in a lone chair in the audience. As everyone guffawed, Rene sat emotionless.
Around 2009, Nina’s mother began to change. While she was always known as a smart, witty, and attractive woman, she started overeating and would go into trance-like states. Always the first to laugh at the family’s relentless jokes, now she didn’t respond. A schoolteacher for decades, she began to snarl at kids and say things like, “I’ll break your fingers if you do that again.” It was a line that could have easily been a joke, accept it wasn’t.
Rene began to say other things as though she were someone else. She talked about sex to people she met on the street. Always one to compliment other women on clothes or shoes, now she’d tell them they were fat as they stood right in front of her. She began to eat all the time, and even steal and hide food. She gained twenty pounds. Sarcasm by others went unnoticed, where she used to respond with witty comebacks. It was as though Rene Cartolano had disappeared, and someone else had taken over. And someone had.
Confused and often embarrassed by the new Rene, the family spent months researching possible brain afflictions that could be causing her behavior. Finally, they got a diagnosis. While there are many types of dementia, Frontotemporal dementia (FTD) is the most common neurodegenerative disease diagnosed before age sixty. It is centralized in the frontal lobe of the brain, a region that is partially responsible for behavior and emotions. While a disease like Alzheimer’s usually affects older people, and is detected when memory issues begin to arise, FTD literally robs people of their personalities first, and like early onset dementia, it occurs in early ages between 30-50. It’s estimated that 10-15% of people with dementia have this type, but don’t know it.
After decades of new research, neuroscientists now have a better understanding of FTD, and the roles different parts of the brain play in how it shapes our personalities. Yet it remains one of the less known cognitive diseases. Dr. Bruce Miller, one the world's foremost researchers on frontotemporal dementia, is leading the science in FTD research. At his lab at the University of California, San Francisco, he and his team are doing revolutionary studies on the two main forms of FTD. In the speech variant, where speech is affected, patients struggle to find words, often pausing in mid-sentence as the words vacate the thoughts. In the behavioral variant, like Rene Cartolanos, personality, empathy, and judgement are attacked. People might start stealing things or do activities that are dangerous to themselves and others and not realize they are doing anything wrong.
Miller’s work shows regions in the brain that have atrophied from FTD and discovered that the amygdala, orbitofrontal cortex, superior temporal, and fusiform cortex regions show the most tissue damage. Also called the perception network, this region is responsible for responding to social cues, eye contact, facial expression, as well as body gestures. Sarcasm response – something that Rene Cartolano stopped reacting to - relies on all these cues, yet FTD also affects the affiliation and aversion networks, which motivates a person to want to have connections with others and experience rewarding emotions during social interactions. FTD patients also begin to trust strangers and possibly unsafe individuals, often giving out personal information, like bank account numbers.
Bolero As A Score For FTD
“We used to think dementias hit the brain diffusely,” Miller says. “Nothing was anatomically specific. That is wrong. We now realize that when specific, dominant circuits are injured or disintegrate, they may release or disinhibit activity in other areas. In other words, if one part of the brain is compromised, another part can remodel and become stronger.” He reminds us the composer Ravel also suffered from a brain disease with symptoms that were identical to FTD.
Perhaps there is no better musical score to represent FTD than Bolero. Ravel composed Bolero in 1928, when he was fifty-three, when errors in his musical scores and spelling began to present. “Bolero is an exercise in compulsivity, structure and perseveration,” Miller said. It elevates with no key change until the 326th bar. Then it accelerates and implodes in a finale. It changes between two main hypnotic themes, repeating the themes eight times over 340 bars with layers of instruments. Yet the score soars methodically with two simple alternating staccato bass lines.
Miller says Ravel was probably in the early stages of FTD when he was working on Bolero. The disease would have altered circuits in his brain, changing the connections between the anterior and posterior regions, resulting in a deluge of creativity. Because circuits in the brain become reorganized in FTD, some patients develop incredible creative abilities. Miller has witnessed FTD patients suddenly become gifted painters, pianists, or engage in skills they never did before as their disease progressed.
A Loss Of Empathy
In FTD brain scans, a deep blue indicates a loss of tissue, and in some scans, almost the entire frontal lobes are blue. “When we see loss of tissue in that brain region, we know people have lost their interest in life, their drive. They do less, they care less about other people,” says Miller. That loss of empathy, Miller says, can trigger dangerous, impulsive, even illicit behavior. Then there are the stories of fathers left watching their children who suddenly lose the ability to care about them, or CEO’s who start walking around their office with their pants down. To the patient, they are not aware that anything has changed, yet to the spouse, it’s as though a light switch was turned off. Miller says because so many cases of FTD are often misdiagnosed as a mental illness like schizophrenia, it can take years and many brain scans to get a correct diagnosis.
The Soul Of Humanity
Miller often says FTD attacks people at the very soul of their humanity. “This is profound as anything that can happen to a human being. It robs us of our very essence of our humanity, of who we are,” he says. It is a devastating financial burden for families, too, as the cost of caring for someone with FTD is astronomical. The outcome of FTD is always death. As FTD erodes the brain, it begins to cause organs to shut down. Swallowing becomes difficult and eventually impossible. Yet Miller is optimistic, leading the promising research both in his lab and by NIH- funded grants to scientists around the globe. “Suddenly we have interventions and research that are going on that give me great hope,” he says. “I'm hoping in the next five years that we will have very powerful therapies in certain variants of frontotemporal dementia that may stop it cold.”
Patients in memory care often ask to go home. Some believe they are still home, in the place they have always lived. Some ask for their dogs, some for their mothers or fathers or siblings who have long since died. For caregivers to explain constantly throughout a day that this is where they live now is not comforting to the dementia patient. To try and ease this distress, many memory care facilities have installed prop bus stops. When a patient asks to go home, the caregiver walks them to the bus stop, where they sit and wait for a bus that never arrives. Sometimes they wait for hours, sometimes they fall asleep. Eventually, as is the course of dementia, their thoughts fade like sunsets, and they forget what they are doing at the bus stop. They are gently led back to their rooms to prepare for sleep. Perhaps when they dream, they dream of a bus that finally came. When they wake in the morning, the first thing they will say is that they want to go home.
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