October 25, 2019
When you hear the word “dementia,” what comes to mind? For most people, probably an image of someone in their 70s or 80s who is suffering from debilitating memory problems. But there is another form of dementia that you’ve probably never heard of: frontotemporal degeneration (FTD), also called frontotemporal dementia or frontotemporal lobar degeneration (FTLD).
“It’s very different from the idea people have in their minds of what dementia is,” Edward D. Huey, M.D., associate professor of psychiatry and Neurology at the Taub Institute for Research on Alzheimer's Disease and the Aging Brain at Columbia University Medical Center and member of the Medical Advisory Council for The Association for Frontotemporal Degeneration (AFTD), tells SELF.
That’s why it’s especially important to be aware of what FTD is and how it can present. Just this week, the AFTD hosted its annual Hope Rising Benefit, raising over $2 million to support patients and caregivers in the community and fund research. Here’s what you should understand about this little-known health issue.
1. FTD refers to a group of brain disorders that cause dementia.
Dementia is a serious loss of cognitive abilities that interferes with a person’s capacity for functioning on a daily basis, the National Institute of Neurological Disorders and Stroke (NINDS) explains.
Alzheimer’s is the leading cause of dementia, and it’s also the one most of us know about. But up to 10 percent of all cases of dementia may be caused by FTD, which is actually the umbrella term for a group of different brain diseases. FTD is thought to affect between four to 15 out of every 100,000 people in the United States, according to the AFTD.
FTD occurs when neurons in the frontal and temporal lobes of the brain die, causing these parts of the brain to atrophy or shrink. This prompts problems with the thought processes and behavior controlled by these lobes, according to the NINDS.
2. FTD is the most common form of dementia in younger people.
According to the NINDS, FTD is the number one cause of dementia in people under age 60. (It seems to affect men and women in equal numbers.) About 60 percent of people with FTD are between the ages 45 and 64, the NINDS says. “FTD basically peaks in middle age,” Dr. Huey says, “as opposed to Alzheimer’s, where your chances of getting it go up as you get older.” However, FTD onset can start as early as someone’s 30s or as late as their 90s, according to the NINDS—so it really spans the age spectrum in that sense.
3. The most common form of FTD causes changes in behavior and personality.
While Alzheimer’s primarily affects memory, that’s not the case with FTD. The most common kind of FTD is called behavioral variant frontotemporal dementia (bvFTD), per the National Institute on Aging (NIA). “The most common thing I hear is, ‘This is just not my husband, this is just not their personality,’” Dr. Huey says. “People start doing things that are out of character.”
The result is a long, varied list of potential symptoms related to behavioral changes, according to the NINDS, like apathy, distractibility, repetitive or compulsive behavior, decreased energy and motivation, changes in food preferences, increased interest in sex, neglect of personal hygiene, and feelings of agitation or emotional blunting. FTD patients may also experience a lack of empathy, a lack of social tact, loss of insight into themselves and others, and disinterest in people they used to care about. For instance, “Someone might lose all interest [in] seeing their new grandchild,” Dr. Huey says. “And their interactions with people become kind of bizarre—they talk too loud, talk too soft, stand too close, stand too far.”
Less commonly, people with bvFTD show symptoms involving language disturbances. People can find it difficult to speak and understand others, and this can occur separately or in conjunction with the behavioral symptoms, according to the NINDS. While estimates vary, Dr. Huey says about 25 percent of patients present with this variety.
4. FTD can be extremely difficult to diagnose for several reasons.
First, many of the symptoms don’t seem medical in nature. “These are not symptoms where you would automatically recognize, ‘We need to go to a doctor because there's a medical problem,’” Susan Dickinson, CEO of the AFTD, tells SELF. And the sheer variety of symptoms means there is not really a classic presentation. There’s also no one single test doctors can use to diagnose the illness, the Mayo Clinic says. Instead, they may need to rely on a battery of exams, such as memory testing and brain scans.
Another huge issue is that the person with FTD is unlikely to recognize these changes in themselves and go to a doctor. “The behavioral variant almost always impairs awareness [in the patient], so they don’t think anything’s wrong and they don’t seek evaluation,” Dr. Huey explains. “So most patients we see, it’s the families [bringing] them in.”
Even when someone is brought to the doctor, physicians may lack awareness of the condition, Dr. Huey says. Since some of these symptoms can also occur with other conditions that are much better known, people are often misdiagnosed. “A lot of patients get misdiagnosed with a psychiatric disorder, often depression,” Dr. Huey says. “It can be a really arduous journey for families of going to 10, 20 different specialists before you get somebody who will really listen and who knows about FTD and who can connect the dots,” Dickinson adds.
5. The cause of FTD is unknown, but there is a strong genetic component.
We don’t know what causes the majority of FTD cases. But about 15 to 40 percent of cases can be linked to a genetic cause, and people with a family history of FTD are more likely to have it, according to the NINDS. In fact, a family history of FTD is the only known risk factor, the Mayo Clinic explains.
Scientists have identified several different genes in which a mutation can lead to FTD. Some of these genes are also linked to the motor neuron degenerative disease ALS (amyotrophic lateral sclerosis), which is why the two often co-occur, per the NINDS.
6. There is currently no treatment for FTD.
One of the worst things about FTD is that it progresses steadily and often quickly. We don’t have treatments to prevent, slow down, or cure FTD, according to the NINDS. Treatments like antidepressants can help manage symptoms by reducing behavioral problems, the Mayo Clinic notes, but that’s more of a stopgap in the absence of better options.
Usually, managing the disease requires a team of specialists—such as speech, physical, and occupational therapists—to provide support, address various symptoms, and improve quality of life for both the patient and their caregivers, the NINDS explains. In its end stages (which can arrive in as little as less than two years or take longer than 10), the disease starts looking similar to the end stages of Alzheimer’s, where the person loses the ability to perform the basic functions they need to survive, like eat, drink, or move. The patient often requires 24-hour care and is at a much higher risk of potentially life-threatening complications like pneumonia.
7. FTD can be incredibly hard on families.
“When you're dealing with a rare disease like this, one of the most definitive features is how isolating it is for the people living with it every day,” Dickinson says. “Often, caregivers and spouses will say one of the hardest things is that they’ve lost their loved one. [That individual] isn’t there for you, and they’re not your partner in this process,” Dr. Huey adds.
That’s why one of the AFTD’s primary purposes is “to create a community where people who are diagnosed and their families feel understood and less isolated,” Dickinson says. To that end, the AFTD offers 100 support groups in 32 states, a wealth of online resources, a toll-free helpline (1-866-507-7222), and various online and Facebook support groups. “Just finding a place where people understand what you’re going through is a huge help. People can share their experiences and challenges, learn strategies that are helpful from each other, and find professional supportive services [and resources],” Dickinson says.
The AFTD also hosts conferences and connects people without support groups in their area to one another. “We might know of three other people who do live near you who are in the same boat,” Dickinson says. “So we can connect caregivers one-on-one to be a friend to listen and understand and maybe provide some guidance.”
8. Research and awareness are gaining momentum.
Most people haven’t heard of FTD, which is why it’s important to spread the word so that families and doctors alike are better able to recognize the condition it when they see it, Dickinson says.
The good news here: “I think FTD has more awareness in the funding and research world than in the general public,” Dr. Huey says.
“There are several very promising avenues of research right now,” Dr. Huey says. “We’re learning a lot.” For example, a group of academic medical centers in the United States and Canada called Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) is currently seeking volunteers for a longitudinal study that will collect clinical and genetic data from patients and their families to help us learn more about the disease.
One huge research priority is identifying biomarkers and neuropsychological screening tools to help diagnose people with greater accuracy and speed, per the National Institutes of Health. Dr. Huey is particularly excited about the strides being made to develop treatments targeted at specific genetic abnormalities. He says these experimental treatments will probably start to enter clinical trials in the next few years. And because of the overlaps between FTD and other forms of dementia like Alzheimer’s, Dr. Huey is hopeful that advances in diagnosis and treatment for one condition may translate to the other.
With all of these efforts, “We’re really working towards a more hopeful future for families facing this truly devastating disease,” Dickinson says. “One where there are drugs, there is a diagnosis, and there is a broader awareness and understanding in society that not all dementia is Alzheimer’s.”