How a beautiful symptom of a brutal dementia informs our understanding of creativity

Carol Spence’s vision is flooded with green. As her brush swoops right and swishes left, she’s first transported to a lush spring meadow, then to the canopies of a rainforest — every stroke bringing her to another verdant utopia.

Until she hears her daughter scream, “What are you doing?!”

Suddenly, she’s in the in-law suite of her daughter’s home. She limply holds the brush that had teleportable powers but a moment ago. In front of her is an antique sewing table that belonged to her mother, smeared with green paint.

“She’d just painted this beautiful antique the ugliest, brightest green you could imagine,” recounted Spence’s daughter, Paige Spence-Berthiaume, in an interview with STAT.

Spence-Berthiaume quickly seized the brush from her mother.

“What! What are YOU doing?! I don’t understand,” said Spence.

“I know you don’t understand, but you cannot paint this table,” replied her daughter.

It wasn’t boredom, or even resentment, that compelled Spence to give this family heirloom a discount makeover; it was a slow but steady death of the neurons in her temporal lobe.

Spence, 63 at the time, had frontotemporal dementia or FTD, a neurodegenerative disease in the frontal and temporal lobes of the brain. These areas control key aspects of who we are — from the decisions we make to our ability to speak — and as they start to decay, patients struggle with behavioral issues and communication. But a small percentage, like Spence, also experience an explosion of artistic creativity. Still, the disease is incurable, and diagnosis marks the start of an agonizingly long goodbye.

FTD is the most common dementia in people under 60 years old. Estimates suggest that 50,000 – 70,000 people in the U.S. live with the disease. But due to the many ways the disease can present, experts believe this is a severe undercount.

“Most people acquire a series of misdiagnoses,” said Susan Dickinson, chief executive officer of the Association for Frontotemporal Degeneration. “It’s most often either a psychiatric diagnosis,” such as depression or bipolar disorder, or other neurodegenerative diseases like Parkinson’s or Alzheimer’s disease, she said.

The disease is caused by a lethal buildup of abnormal proteins in brain cells. Proteins like TDP-43 and FUS, two common pathological hallmarks of FTD, normally regulate how RNA is processed in the cell nucleus. But in FTD, aberrant mutations and modifications cause the proteins to misfold and aggregate in regions where they shouldn’t, disrupting proper RNA processing and interfering with cells’ normal activities, causing them to die. The tau protein, whose buildup is most commonly associated with Alzheimer’s disease, is also implicated in FTD. It typically helps assemble and stabilize the long cable-like extensions of neurons that send electrical brain signals. Like TDP-43 and FUS, mutations and modifications to tau disrupt its function and cause it to aggregate, making neurons die. Close to two dozen other genetic mutations have been identified in FTD.

If neurodegeneration begins on the right side of the frontal or temporal lobes, patients tend to struggle with compulsive or inappropriate behaviors and emotional regulation. These symptoms include loss of empathy for others, disregard toward social and legal norms, loss of drive, overeating, and repetitive behaviors like tapping a pencil incessantly. But if it starts on the left side, patients have difficulty with understanding and communicating language. They lose the meaning of words, leading them to speak nonsensical gibberish, or lose the ability to form words, making it difficult to converse. As the disease progresses, behavioral and linguistic symptoms eventually overlap.

“It’s really a brain disease that affects people in sometimes strikingly different ways depending on which parts of the brain it starts in,” said Bradford Dickerson, director of the Frontotemporal Disorders Unit at Massachusetts General Hospital and Spence’s neurologist.

Sometimes though, FTD patients can exhibit an additional, peculiar symptom: a flourish of artistic creativity.

Spence was one of these patients. As her dementia started, she began dedicating a lot of her time to art. Her artistic talents weren’t new, as she had crafted intricate miniature dolls to display and sell at craft fairs and galleries before her diagnosis. But she had stopped making dolls, and showed an entirely new interest in painting — regardless of whether a canvas was available. After the antique sewing table makeover mishap, Spence-Berthiaume stocked her mother up with proper art supplies and let her imagination run free.

“It was an obsession. It filled her day, the painting,” Spence-Berthiaume said. In her dementia, Spence would spend most of her waking hours putting paint brush to canvas — painting the beach she lived by, plants and animals, and portraits of herself and loved ones — all with a vibrant, two-dimensional charm.

Spence experienced a rare phenomenon in FTD that causes patients to become heavily preoccupied with their visual surroundings. If their brain wiring is just right, they turn to art — be it painting, music, dance, or design — to express these newfound fascinations.

Bruce Miller, director of the Memory and Aging Center at the University of California, San Francisco, first discovered this phenomenon in the 1990s. “Pretty early on in my work on [FTD], I saw a gentleman who was a banker who’d never been interested in painting,” he said. But in his dementia, he suddenly “started painting and became very visually obsessive. … Within two years I’d seen 12 people … who had been visually or musically creative, and all in the setting of [FTD],” Miller said.

There’s an important distinction between those who practice art therapy in FTD and those who experience the visual artistic creativity phenomenon. Adit Friedberg, a research fellow at the Memory and Aging Center at UCSF who works with Miller, said the phenomenon typically manifests in one of three ways: Patients may develop a sudden affinity for art in their dementia that had not existed before, or they show a dramatic change in the type or style of art they produce, or they dedicate an enormous amount of time to art, creating a sheer plethora of it.

In a study that Friedberg and Miller recently conducted, only 17 of 689 FTD patients they surveyed met any of these inclusion criteria, or just 2.5% of the cohort. “We’re talking about a gain of function in a setting of neurodegeneration,” Friedberg said, making the phenomenon pretty hard to come by.

But it may be more common in patients with a type of the disease, called semantic FTD, which makes up about 20% of all FTD cases. This variant targets the temporal lobe — the area of the brain that processes language — causing patients to have difficulties recalling and understanding words. Early in his work, Miller observed that the brains of patients who experienced visual artistic creativity initially experienced neurodegeneration in the left temporal lobe, but the frontal lobes were largely spared. In Miller and Friedberg’s new study, 7% of patients with semantic FTD exhibited this creativity, a number which study authors believe is remarkably high.

The temporal lobe “plays a key role in storing relationships between concepts,” Friedberg told STAT. It’s responsible for our semantic memory, or our explicit knowledge of the world through facts and processes. For instance, our semantic memory contains information about dogs — from what they are to how different breeds look and behave. Friedberg speculates that as the temporal lobe degenerates in these patients, such relationships and concepts become frayed, and that “may contribute to generating novel and inspiring visual art.”

Miller, who has seen dozens of FTD patients with visual artistic creativity, has noticed certain trends in their artwork. “Colors are very prominent in the pictures — bright colors. I think the ones most commonly used are purple and yellow, but sometimes really verdant greens. So very bright, realistic or surrealistic in general,” he said. Patients tend to depict concrete objects or things they can see over abstract concepts. Many also experience a compulsive need to paint, which can lead to repetitive designs or color patterns, Miller said.

Dickerson believes this preference for bright colors can be explained neurologically. Healthy people usually “have an idea that they’re trying to convey conceptually through their artwork,” he said. “That degrades in people with this disease…so they often get very attracted to what you might call lower level visual features of their environment, like contrast or patterns,” he said.

While painting is the most common medium of expression, patients could take up other forms of art, like sculpting, playing music, or even building a garden. “One of my favorite artists…is a gentleman who welded, and he did these beautiful insect-like birds,” Miller said. In the subset of FTD that affects language, called semantic FTD, “you lose the knowledge about differences between animals. So [he made] these prototypic evolutionary-like figures.”

Another individual who experts speculate experienced this phenomenon was Maurice Ravel, a French composer from the early 1900s. His composition, “Boléro,” is a fifteen-minute song that features one melodic clause over and over again, which could be a symptom of the compulsive repetition that’s sometimes observed with FTD patients. Ravel also lost the ability to speak before his death, another hallmark symptom of FTD. Of course, it remains entirely possible that Ravel just wanted to create an evocative piece; without his brain, we’ll never know for sure.

Perhaps the most well-known patient who experienced visual artistic creativity was Anne Adams, a biologist at the University of British Columbia with a nonfluent type of FTD that would cause her to lose her speech. Adam’s husband, Robert Adams, said that she “took [art] up with a vengeance after [our son] had his [car] accident. She just decided she was going to devote a lot of time to it.” Their son quickly recovered, but Adams’ obsession with art never wavered. Eventually, she abandoned her scientific career to pursue art full-time.

Adams’ artwork reflected the passions of her life. She painted symmetrical patterns of biological creatures, a color-by-number representation of the first 1500 digits of pi in honor of her mathematician husband, and visual interpretations of her favorite compositions of classical music.

One such painting is a bar-by-bar representation of Ravel’s famous composition, punnily titled, “Unravelling Boléro,” though Adams had no knowledge of Ravel’s suspected FTD at the time of creating her art piece. Adams enjoyed puns early in her dementia, according to her husband; another one of her paintings based on Wolfgang Mozart’s “Rondo alla turca,” heavily featuring the color turquoise, was named “Rondo alla turquoise.”

Now, research has revealed a neurological basis behind this artistic creativity. Friedberg and Miller analyzed the brains of the 17 artistic FTD patients they identified, and observed a striking trend in their scans. In these patients, the left temporal lobe had deteriorated as expected, but concurrently, regions in the back of the brain responsible for visual processing had hyperactivated. This activity was not observed in healthy brains or patients with FTD who did not experience artistic creativity.

Researchers still don’t fully understand what causes this activation, but Miller believes the brain regions involving language and visual art are inversely connected. Because brains dedicate so much energy to processing language, it’s possible that other areas, like those that process visual inputs, may be neglected.

In a society where language is so dominant, people with “dense neuronal concentrations in the back of the brain that could produce beautiful art…process other things” like language, Miller said. “With these degenerative diseases, suddenly areas of the brain that were neglected or deactivated most of the time” are allowed to activate, he said.

The brain scans also revealed a surprising relationship between visual processing and right hand movement. As the size of the brain region involved in visual processing grew, so too did the size of a motor region representing movement in the right hand. According to Miller, this “suggests that the use over and over again of the right hand, with visual stimuli, actually led to, we think, real changes in the structure of the motor strip that was necessary for painting,” he said.

More broadly, Friedberg and Miller’s findings suggest that physical changes in the brain might awaken a person’s creativity. It isn’t just an innate trait that some people are born with; creativity can be cultivated.

“In some people it’s very evident that their brain circuitry is wired in a certain way that really drives them to do creative things,” Miller said. But by practicing creative skills, “we now know the brain will change — you can change the synapses, you can change the density.”

Visual artistic creativity is a beautiful symptom that can emerge in some patients with FTD, but it doesn’t slow disease progression. While Spence’s artistic output exploded, slowly but steadily, her vocabulary dwindled.

“We would go to soccer games on a weekend morning, and [my kids] would come off the field and she’d point at them and tell them how bad they were,” said Padgett Berthiaume, Spence’s son-in-law. “She didn’t mean it…it’s just the words that she could say,” said Berthiaume. Spence would misplace other words for those she’d lost. She’d frequently say “I have to go do artwork,” when she had to use the bathroom.

Spence-Berthiaume also reflected on how aspects of her mother’s personality changed over time. It was incredibly difficult to see someone who, before her dementia, “cared about how she dressed and just was so kind to everyone…[start] burping in public, or spitting on a plate, or stealing,” she said. “I would get so mad at her. And that was really hard, to have so much anger towards somebody who couldn’t control it.”

Eventually, signs of Spence’s deterioration became apparent in her artwork. Spence-Berthiaume noted how over time, her mother’s paintings devolved almost in parallel with her mental state. “The life of the subject that she was painting became more and more flat, just like she was,” she said. “Eyes on the characters were really detailed, and then by the end, they were just black empty eyes.”

As Spence’s FTD progressed, neurodegeneration spread from the left to the right temporal lobe, a region critical in recognizing faces and detecting emotions. Spence’s self-portraits, which depict increasingly hollow facial expressions over time, are “not surprising when you think about the systems that are damaged in [FTD] that are related to social and emotional processing,” Friedberg said.

But perhaps the most tragic aspect of FTD is that, because the disease affects a person’s behavior and language, family and friends can never know how a patient is truly doing, or how they perceive the loss of their own identity.

“[FTD] doesn’t just steal somebody’s memory. It’s stealing their personality and their ability to communicate with the people they love…It [steals] what makes us who we are,” Dickinson said.

After several years of prolific artistic expression, Spence lost her ability to paint. She passed away in December of 2022, 11 years after her diagnosis. Her family still keeps hundreds of her paintings in their home.

“I’ve got one closet that’s full…and then in the attic, I haven’t been up in a while, but [her artwork] has got to take up the size of an average bathroom,” Spence-Berthiaume said.

Similarly, Anne Adams, who was diagnosed with FTD in the early 2000s at age 62, lost most of her ability to speak by 2004. “She had been a very articulate person — she was an academic,” recalled Adams’ husband. “We’d frequently have interesting conversations earlier on, [but] those kind of petered out because she couldn’t speak after a while,” he said. Adams passed away in 2007.

Currently, there are no cures or medical treatments for FTD. “Those brain cells are dying, [and] we don’t yet have a way to change that fact,” Dickinson said. “Our goal right now [is] to retain quality of life for as long as possible.”

But hope may be on the horizon. Clinical trials for genetic variants of FTD are underway, such as a monoclonal antibody that works to sustain levels of GRN, a protein involved in cell survival and inflammation that is usually depleted among high levels of TDP-43. Gene replacement therapies for GRN are also in clinical development. Other modalities include drug repurposing of a small molecule currently used to treat amyotrophic lateral sclerosis, another neurodegenerative disease, and intracranial current stimulation.

The FTD community is “tremendously excited about the recent approvals for Alzheimer’s treatments,” said Dickinson, as they could foreshadow a similar future for FTD. “Momentum is building there. And we are very hopeful that that will stimulate more investment in FTD by biopharma companies,” she said.

As rare as it is, visual artistic creativity may be more than just a beautiful symptom of an otherwise brutal disease. It helps patients hold onto their personhood for as long as possible, and it helps families celebrate and remember their loved ones.

Today, a painting of a cat hangs in the office of a hospice team that cared for Spence. When they look at it, they smile, and think of her.