At the time of his wife’s death, John Carter was a shy and conservative 52-year-old man whose most distinguishing traits were an uncanny ability to remember numbers, a two-stroke golf handicap, and a profound devotion to his wife. So the prolonged grieving that followed her death surprised no one. But when John decided to leave his brokerage firm and become an artist, his family and friends were shocked. To their knowledge, John had never before shown any interest in art whatsoever; indeed, he might have been the only man in his Connecticut suburb who had never visited an art museum.
To his college-age twin daughters, John explained that he was feeling bombarded by colors and sounds. His life, he said, had to change. He moved to a simple loft and started dating art students, many younger than his daughters. He gave up eating meat. He began wearing loud purple shirts and yellow pants, attire that seemed silly to those who knew the former John. He began to fill canvases with streaks of purple, yellow, red, and green. They impressed no one. Still, no one doubted that John was in great mourning, and if the price of being an artist was living with an extreme intensity of feeling, then he was paying that price.
John began to tell friends of his new ability to see not only colors but sounds. Sometimes, he explained, he felt open; during these periods, colors bounced through his head like notes of music, and each shade of the rainbow vibrated with a clarity that could bring him to tears. On these open days, John was warm and mellow, spending many hours painting in solitude, leisurely capturing on canvas the beautiful hues and images of flowers and animals that raced through his mind. When he felt closed, though, he was sad and irritable, grimacing at the bark of a dog or an intrusive shaft of sunlight. At such times John painted with a desperate ferocity, rapidly splashing the canvas with colors, hoping to break through to a feeling of openness.
No one can remember exactly when John’s paintings began to appeal to the eye, but it seemed to happen around the same time that he began to have trouble remembering the meaning of words. At first people assumed that John was kidding when they would say a common word like key, and John would repeat back, Key, key, what’s a key? With time, John comprehended fewer and fewer words, and the words he chose to convey his own thoughts became more and more simple. John had been an articulate, thoughtful man. But within three years there was little trace of his formerly rich speech patterns. He conversed in short, simple phrases, like I feel open or I feel closed.
As his speech became more impoverished, John grew more flagrant in scorning social rules. One day the police called his daughters to bail their father out of jail after he had appeared nude in a public swimming pool. Other days, John walked the streets of the city, intentionally bumping into and berating strangers. His temper grew so vicious that he began kicking his dog. His daughters began finding pens that John had swiped, and they noticed his tendency to steal tips off restaurant tables. Soon John required caretakers around him 24 hours a day to prepare his meals, wash his clothes, calm his moods, and keep him out of jail. His compulsions grew stronger. He often dragged his caretakers past banks, phone booths, Laundromats, and restaurant tables, hunting for dropped coins.
Unexpectedly, as John’s social skills and language abilities eroded, his visual senses became more acute. He found coins that no one else would have found, and he became exquisitely sensitive to sudden changes, such as subtle shifts in the weather. John devoted these new visual skills to his painting, spending hours in front of a canvas perfecting every line, often using the same purples and yellows that he favored in his clothes.
Over the years the complexity of John’s art increased. His early paintings had featured simple colored lines; now he was exploring interesting rounded shapes. For several years, he painted richly colored flowers, boldly laid over a stark monochromatic background. At age 58, some five years into his illness, John spent three weeks in Hawaii, where he felt open almost every day. After returning home, he re-created from memory the beautiful birds of Hawaii, usually in purple and yellow. These tropical birds—perhaps his most attractive pieces—evoked the feeling of another world. Several won prizes at local art shows. Yet this accomplishment seemed to give him little pleasure, and by the time he received these awards, John was a shell of what he had been. His daughters sought medical opinions about his condition; diagnoses ranged from Alzheimer’s disease to depression to schizophrenia. No medication helped.
John’s language skills continued to falter and his violent outbursts grew more frequent. His paintings became less realistic. One series featured elephants, pigs, and dogs with rounded, slipperlike feet and distorted facial features. Next came his doll paintings. The images were bizarre and faintly sexual—one image featured a doll emerging from the other’s abdomen. The only element they shared with his lovely bird paintings was the lavish use of purple and yellow. John’s open times were now rare, and he was frequently plagued by closed periods during which he could not paint. Sounds and colors had been a source of joy; now they brought him only pain.
It was about eight years after he began his artistic life that I first met John. I am a neurologist who specializes in behavioral disorders, and John’s daughters brought him to me in the hope that I might shed some light on his condition. When John arrived for his appointment, he was dressed in a long-sleeved purple T-shirt and yellow pants. He was tall, thin, and oddly handsome, but his drawn face and gray hair made him appear all of his 61 years. During his visit, John never smiled nor showed any reassuring or responsive facial expressions. His voice was soft and flat, and he was so remote and distant that it was hard to empathize with him. John repeatedly stated in a monotone, Doctor, can you help me? I feel closed. I’m closed to colors and sounds. At one point, he said quietly that he was tired and abruptly lay down on the floor. He did not rise for several minutes. After he stood up, I began his examination. John’s coordination was excellent, and he moved with the agility and lightness of an athlete. He showed no deficits on the basic neurological exam, which tested his senses, motor skills, and responsiveness. I then performed a detailed assessment of John’s language, intelligence, memory, decision-making abilities, and visual and spatial skills. John’s language consisted of a series of pat phrases, and he kept repeating, Doctor, can you help me? Last week, every day I was closed to color and sound. He could not name simple objects like a watch or pencil and failed almost all memory tests. Yet he was able to re-create from memory more intricate designs than any other patient I had ever examined, and he copied complex designs with extraordinary precision. He performed outstandingly on these two visual tasks but failed all the others.
We then performed imaging studies of John’s brain. The human brain is divided into two hemispheres; each hemisphere houses four different lobes, or regions. The frontal lobes, of course, lie in front; the occipital lobes lie in back; sandwiched in between are the parietal lobes, on top, and the temporal lobes, below. The images of John’s brain revealed diminished function in the front of his temporal lobes—a region that is involved in many social and perceptual tasks. Yet the rear portions of John’s temporal lobes—and his parietal lobes—were normal. That helped explain John’s unusual condition. The rear part of the temporal lobe is involved in our ability to visualize images; the rear part of the parietal lobe is involved in our ability to put those visual images on paper. Both skills are usually lost in Alzheimer’s patients. In John, however, these areas were either normal or improved in function. These images confirmed what John’s progressive deterioration already suggested. John suffered from frontotemporal dementia, a degenerative disorder that causes loss of brain cells both in the frontal lobes and in the front part of the temporal lobes. The disorder is sometimes inherited via a defective gene from one parent or the other, but patients are usually spared signs of the disorder until they reach their fifties. Because the brain-cell loss occurs in parts of the brain that help regulate social behavior, personality changes are the most common manifestations of the illness. Patients tend to become either uninhibited or apathetic.
Until recently, most of these individuals were thought to suffer from a primary psychiatric illness—such as schizophrenia or bipolar disorder—while still others were misdiagnosed as having a degenerative condition that often accompanies aging, such as Alzheimer’s. Unfortunately, like so many degenerative diseases, there is no cure for frontotemporal dementia. When I explained the disease to John and his daughters, I told them why I thought his intense interest in visual images was probably directly related to his condition.
Most patients with frontotemporal dementia do not become more artistic as their disease progresses. John was an exception. Some of the explanation for his artistic renaissance will remain hidden forever, but it was probably fueled by a mix of talents, experiences, and brain circuitry unique to John. Yet an anatomic anomaly in his brain imagery held a very strong clue. Unlike those of most patients with this disorder, John’s frontal lobes, the regions that govern our ability to exercise complex thought, judgment, and planning, were spared. His loss of brain cells was restricted to the front part of the temporal lobes. This unusual pattern of brain-cell loss probably contributed to John’s curious combination of cognitive impairment, language deficits, bizarre behavior, lack of inhibition, and artistic creativity. The brain contains a series of excitatory and inhibitory circuits; when one circuit is stimulated, other areas are turned off. This reciprocal inhibition prevents us from activating competing brain circuits simultaneously. Imagine trying to type a paper, remember a list of words, and compose a poem at the same time. Clearly, when we try to do so, the efficiency of each competing brain circuit decreases. This concept of reciprocal inhibition may help explain John’s sudden burst of creativity.
The front part of the temporal lobes houses our social brain. When the social brain functions well, we succeed with those around us, but when it is dysfunctional, socially unacceptable and bizarre behavior emerges. In contrast, the rear part of the temporal lobes—the posterior temporal lobes—helps us interpret and make sense of the world’s visual attributes. Localized here are specialized cells that respond to shape, color, movement, and faces. When an artist imagines the visual images that he or she puts into a picture, the posterior temporal lobes become active. And skills housed in the adjacent parietal lobes help the artist place that image on paper.
In John’s case, dysfunction in the front of his temporal lobes led to progressive deterioration of his well-developed social inhibitions and restraints. At the same time, it heightened activity in his posterior temporal lobes, leading to a continuous flood of visual imagery that preoccupied, and often tormented, his waking moments. In other words, the inhibitory fibers that ordinarily turn off stimulation from the visual brain seemed to have stopped working, perhaps allowing the artist in John to wake from dormancy. That, at least, is what I believe is happening. What I do not understand is why John’s condition prompts him to alternate between feeling open and shut down. I can only speculate that his mood swings are probably akin to the highs and lows that patients with bipolar disorder experience. These swings may be caused by an inability of the inhibitory and excitatory circuits to reach equilibrium. It may be like a thermostat that has lost the ability to regulate itself.
I continue to see John regularly. Occasionally, I prescribe antidepressants to help lift him out of the gloom that descends on him. At this stage in his disease, he is living at home with a caretaker. He is speaking less and less, and he no longer has any interest in painting.
John’s long obsession with visual images is not common among patients with his disorder. Still, he is not the only patient in whom we have seen artistic ability flower among the ruins of progressive cognitive and social impairment. Joan, a middle-aged housewife, stopped entertaining her husband’s clients and began drawing beautiful scenes she remembered from her childhood. Mark, a businessman, made intricate wax figures and compulsively photographed buildings from many angles. Miguel, a janitor, painted pictures he remembered of Native Americans in New Mexico. All three patients were ultimately diagnosed with frontotemporal dementia. Paradoxically, in all of them, a period of exceptional creativity heralded the beginnings of a tragic disease.