Frontotemporal dementia (FTD) is a specific type of dementia that often affects people at the prime of their life. It can impact language, personality, decision making, and personal interaction. The cause is unknown, but FTD affects the frontal and temporal regions of the brain. These parts are responsible for personality, language, and behavior.
FTD is the most common form of dementia among people under 60. It is also the most misunderstood and often misdiagnosed.
Here are distinct characteristics of FTD that might help you have a better understanding of the disease.
1. It tends to affect a younger age group
The majority of frontotemporal dementia cases occur between the ages of 45 and 64. It can also affect people as young as 21 years old, and as old as 80.
Because many people affected by FTD are still working-age, the disease may cause them to act in a different way. They might make poor decisions about their jobs, relationships, and finances. This can impact and alienate their loved ones, and isolate them at a time when they need others the most.
People don’t expect dementia to be present in younger individuals. Thus, the changes in behavior are often viewed by others as occurring because of personal choice, and not a medical condition.
2. It affects personality, behavior, speech, and/or movement
Unlike Alzheimer’s disease, memory loss does not occur in the early stages of frontotemporal dementia. However, it can occur as it progresses later on.
There are different types of frontotemporal dementia. Determining the specific type may be difficult in the early stages as symptoms, and the order in which they appear can vary.
For example, language-related difficulties are the main symptom of primary progressive aphasia (PPA). These can also appear in later stages of behavioral variant frontotemporal dementia.
Behavioral variant frontotemporal dementia
The most common type, behavioral variant frontotemporal dementia (bvFTD), results in often dramatic behavioral and/or personality changes.
Apathy
Apathy, which may be misconstrued as depression, is a hallmark symptom. These people might lose interest in the things they once loved, lack the drive to perform daily activities. They may also have problems starting activities without help. People with bvFTD tend to have stronger levels of apathy compared to other types.
In addition, a 2020 University of Cambridge study showed that apathy could predict the onset of FTD many years before symptoms start. The study followed people carrying the FTD-causing gene and their relatives without the gene over several years.
Scientists found that people with genetic mutations developed more apathy than other members of their family. Cognitive decline also increased as they approached the estimated age of onset of FTD symptoms. This discovery suggests that the disease can be treated at an early stage.
A lack of empathy
People living with bvFTD may also exhibit a lack of empathy. They may show a cold reaction to loved ones who are ill or in pain. Reading social cues may also be difficult for them, and they may appear to others to be selfish or uncaring.
They may also lose their insight and “filter”, and blurt out things that may be offensive to others. Some may engage in impulsive behaviors like stealing. They may also develop compulsive eating behaviors, or neglect basic hygiene.
Imprisonment and bvFTD
Imprisonment resulting from behavioral-type FTD is a real problem, says subject matter expert Sharon Hall, and co-founder of iCARE Advocacy. Many people living with FTD are in jail when diagnosed. This is tragic, as the disease causes them to lose self-control due to brain degradation, and not because of their personal choices or who they are.
But as their actions often violate norms, rules, and laws, they can get into serious trouble. An increase in education and knowledge of FTD within the legal community is needed to help identify individuals living with FTD so they can receive treatment and care instead of incarceration.
As the bvFTD progresses, people may exhibit difficulties in language and communication, like the symptoms of PPA.
Primary progressive aphasia (PPA)
Primary progressive aphasia (PPA) is characterized by changes in language and communication. People living with PPA may have difficulty speaking, writing, reading, or understanding what is being said.
There are three types of PPA, indicated by the type of language problems that are initially manifested. These are:
- Semantic PPA, where individuals have difficulty comprehending words and recognizing faces;
- Agrammatic PPA, where individuals have trouble speaking;
- Logopenic PPA, where individuals y might have difficulty finding the right words but can comprehend words and sentences.
People living with PPA may exhibit symptoms of bvFTD as the disease progresses.
Movement-related frontotemporal dementia
Two rare disorders associated with FTD primarily affect movement. These are corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP). The prevalence of both CBS and PSP is approximately 5 in 100,000. Their exact cause is not yet known.
CBS is characterized by the progressive loss of the ability to control movement. Its primary symptom is the inability to use one’s arms or hands.
PSP is characterized by difficulties in balance and walking. Some symptoms of PSP are similar to those of Parkinson’s disease. These include slow movement, body stiffness, and the loss of facial expressions.
Behavioral and language-related problems, similar to that of bvFTD and PPA, may develop as the CBS or PSP progresses.
Patients with bvFTD and CBS also typically have deficits in executive function. This is the ability that encompasses planning, problem-solving, reasoning, judgment, and attention.
3. It is often misdiagnosed as a psychiatric illness
Diagnosing frontotemporal dementia can be difficult. Its symptoms overlap with other disorders such as Alzheimer’s disease, Parkinson’s disease, schizophrenia, vascular dementia, or depression.
Because it tends to occur at an earlier age, it can also be confused for psychiatric disorders. Symptoms may also cause misunderstanding and anger among loved ones. They may give the impression that the person is misbehaving.
Statistically, a doctor will see between zero to two people with FTD in their entire career. The average neurologist will only see 5-10 cases in their career. If they do encounter it, many do not have the experience to deal with it.
On average, it currently takes 3.6 years to get an accurate diagnosis. Brain imaging procedures such as computed tomography (CT scan) and magnetic resonance imaging (MRI) can help physicians rule out other disorders or diseases and pinpoint FTD, as they reveal atrophy in the frontotemporal regions.
4. It can be inherited
A 2009 study by the American Academy of Neurology found that 42% of individuals with FTD have a family history of dementia. The rest of those surveyed have cases of FTD that are sporadic. This means they did not inherit the disease from a relative.
Researchers also determined that behavioral problems are more likely to be hereditary and that language-related problems are the least likely to be hereditary.
5. It increases the risk of other illnesses
A person with frontotemporal dementia can live for an average of six to eight years from diagnosis. The duration can vary from case to case. Most deaths related to FTD result from complications arising in its advanced stages.
Pneumonia is one common cause of death in FTD. Those with primary progressive aphasia, a type of frontotemporal dementia that affects speech, typically develop problems with swallowing. This can result in aspiration pneumonia; that is, the entry of materials from the mouth or stomach into the lungs, which causes infection.
Psychological changes caused by FTD can result in the person engaging in impulsive or dangerous behavior. They are also at increased risk of getting fall-related injuries or infections.
6. It is possible to live a full life after a frontotemporal dementia diagnosis
There is no cure for FTD. Still, if sufficient support and person-directed care are provided to those living with the disease, it is still possible for them to live a full life.
In fact, people living with FTD still need a lot of independence and autonomy. They will often have the memory and cognitive function to do activities such as games and gardening on their own. While they may need help to manage their impairments such as lack of control or impulsive behavior, they can keep themselves busy. It’s important to let them do what they want, as long as it’s safe for them, and for others. Do not focus on the limitations or things someone can’t do because of FTD. Rather, consider all the many fulfilling things they can do.
7. It requires a lot of understanding and empathy from others
Difficult behavior resulting from FTD can cause a lot of pain, especially for their loved ones. When a person with FTD makes comments about another person, it might be easy to blame them, and not the disease, for acting inappropriately. Living with these tendencies will require a lot of understanding. This especially applies to their close contacts.
It can also help to be open and educate neighbors, colleagues, and community members about a loved one’s diagnosis of FTD. This will help them to understand, empathize, and be supportive of your loved one as they continue participating in the outside activities they love.
In conclusion
Living with frontotemporal dementia can be difficult for both the person with FTD and their loved ones. Nonetheless, there are many resources and interventions available to preserve their quality of life. These can also make each day a little more joyful and easier for people living with FTD and their care partners. Education, understanding, and positivity are all important.
Researchers and medical practitioners are also working to better understand FTD and to determine more ways to manage and treat the disease.
The Association for Frontotemporal Degeneration is a great resource for knowing more about frontotemporal dementia, how to manage its challenges, and connecting with those who understand.