A Lesser-Known Dementia That Steals Personality

Frontotemporal dementia, unlike Alzheimer's, often hits people in the prime of their lives, and can make them act like a completely different person.
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It was the annual Labor Day tradition for the Savini family, a makeshift version of The Gong Show performed before the neighborhood on a wooden deck stage at their beach house in Massachusetts. In past years, Nicole Savini’s mom and friends dressed up in nightgowns as the housewives version of The Supremes, singing “Stop in the Name of Love” into wooden spoons.

To the Savinis each year, it’s seriously funny business—as if they’re performing at the Apollo, said Nicole, 36, whose humorous background has influenced her sensibilities at The Colbert Report on Comedy Central, where she is a senior producer.

The theme this year involved a tongue-in-cheek skit on global warming, with the family singing rewritten lyrics to “Don’t Worry Be Happy,” complete with a fake weathercast warning of Sharknado’s arrival, as well as the Lobster-pocolypse and Clamageddon. Nicole’s dad, John, dressed up as a shark from Jamaica.

But one person was emotionally absent from the production, though physically present: Nicole’s mom, Kathy.

For most of Nicole’s life, her mother would get into the spirit of “The Gong Show,” dressing up in costume, practicing the choreography and lines. Instead, this year a special seat had been reserved for her among the lawn chairs in the audience, and while everyone else cracked up, Kathy didn’t react.

Five years ago, Nicole’s mother — who had always been an attractive, clever, fun-spirited woman — began zoning out and overeating. She eventually stopped laughing at the family’s wisecracks. Kathy had worked as a librarian at a local school for a decade, but she started snapping at the kids in a manner that was out of character, saying things like, “I’ll break your fingers if you touch that again.” The Savini family, always quick with one-liners, might have found it funny if Kathy had been joking — except she wasn’t.

Nicole’s mother lost her filter. She talked about sex to strangers. She used to be able to dish out flattery. “You look fantastic in that bikini,” Kathy would say, making a woman’s day. Now, she mentioned if she thought someone was fat, right in front of them. Meanwhile, other people’s sarcasm slipped right by her. She just didn’t get the quips anymore.

It took many months, and some Internet research, for the family to get a diagnosis. Her mother’s change in behavior was caused by a little-known disease called frontotemporal dementia, a neurological disorder centered in the frontal lobe of the brain, the part responsible for our behavior and emotions. While Alzheimer’s usually affects older people, and is detected as a person begins to lose memory, frontotemporal dementia causes people to lose their personalities first, and usually hits in the prime of their lives — the 30s, 40s, and 50s.

Over the last decade, new research in patients with frontotemporal dementia and other illnesses, has helped neuroscientists understand more about the roles different parts of the brain play in where our personalities come from.

A study released in October by Dr. Brad Dickerson and colleagues at Harvard Medical School in the Journal of Neurology, Neurosurgery & Psychiatry pinpointed regions in the brain that showed atrophy from frontotemporal dementia and found that those with the most damage to the “perception network” (amygdala, orbitofrontal cortex, superior temporal, and fusiform cortex) also showed the most prominent difficulty responding to social cues, facial expressions, and eye gaze, and had the most trouble interpreting gestures and body language—the kind of cues that sarcasm relies on.

Frontotemporal dementia patients with the greatest deterioration in the “affiliation network,” (involved in motivating a person to connect with others and generating rewarding feelings during social interactions) exhibited the most severe social and emotional detachment, and patients with the greatest damage to the “aversion network” (involved in detecting and avoiding untrustworthy or threatening individuals) became more willing to trust strangers, and in some cases gave away private personal information despite negative consequences. 

Journal of Neurology, Neurosurgery & Psychiatry

On the surface people with frontotemporal dementia might still seem normal, able to hold a job, and talk to people about the weather. But those traits that family and friends know and love—such as empathy, shyness, shame, pride, or wittiness—begin to disappear.

“What it steals are the things we value most as human beings. The desire to tell our family we love them, the ability to control our behavior, the very essence of who we are—our personality,” said Susan Dickinson, executive director of The Association for Frontotemporal Degeneration, a national nonprofit organization based in Pennsylvania.

As the disease invades parts of the brain, patients’ altered behaviors have helped researchers understand how embarrassment comes from the pregenual anterior cingulate cortex. Or how recognizing sarcasm can be traced to structures in the temporal lobes, according to UC San Francisco researcher Kate Rankin, who has studied frontotemporal dementia patients.

Nicole’s mom’s diagnosis was especially ironic, given their close-knit, funny family in Marshfield, Massachusetts. Sarcasm had always been a central element of Nicole’s life, leading her to produce satirical segments for Stephen Colbert’s show, like this one on Maine’s missing scallop gonads, which, today, would go right over her mom’s head.

There are moments now when all Nicole can do is laugh at how simultaneously tragic and hilarious her mother’s illness is. She sees absurdity in situations that her mother can no longer grasp. Lately, Kathy has been obsessed with opening people’s mailboxes. “We keep telling her it’s a federal offense,” said Nicole.

Then, Kathy will open another.

“Mom what is that?”

“Federal offense,” she will reply in a monotone. “Federal offense.”

“People say all the time that comedy comes from tragedy,” Nicole said. “If you can’t laugh, you’re just going to be crying all the time. But it’s an overwhelming disease. You are really helpless. There is no medication, no road map, no end in sight.”

* * *

An estimated 50,000 to 60,000 people in the U.S. are diagnosed with frontotemporal dementia, although experts estimate that many more suffer from the condition but have not yet received a correct diagnosis, according to The Association for Frontotemporal Dementias.

The symptoms are often wrongly blamed on alcoholism, depression, menopause, mid-life crises, stress, or schizophrenia, and patients can go through years of negative tests for other ailments like cancer, strokes, and syphilis before learning the truth about what is actually wrong. People living with frontotemporal dementia, are unaware that they have even changed. But for loved ones, sometimes it’s like living with a stranger.

It affects each patient’s character differently. A proud CEO and dedicated father could one day start walking around with his pants down, or stealing candy from a convenience store without embarrassment or shame. A devoted girlfriend might nonchalantly tell her partner intricate details of past relationships with other men.

Nurse and medical anthropologist Jill Shapira, who works at the University of California, Los Angeles with frontotemporal dementia patients and their families, recounted the story of a husband who had always been loving and kind. One day, his wife fell on the floor. As she was sprawled on her back, he stood over her and asked, “When’s dinner?”

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Erika Hayasaki is an assistant professor of literary journalism at the University of California, Irvine, and a former national correspondent at the Los Angeles Times. She is the author of The Death Class: A True Story About Life.

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