The Early Signs of Frontotemporal Dementia

This article was medically reviewed by Heather Collins, MSN, RN, CNOR, NE-BC.

A loved one undergoing massive shifts in personality or speech can be challenging to work with. You might be wondering if it’s already a symptom of an underlying disease. Frontotemporal dementia (FTD), in particular, may be at play, and identifying ways to slow down its progression can help. In this article, Village Caregiving details what to expect with frontotemporal dementia — its symptoms, how to get diagnosed and what you can do as a family member of someone affected. 

What Is Frontotemporal Dementia?

Frontotemporal dementia is a brain disease that affects the parts of your brain responsible for behavior, speech and memory. “Frontotemporal” stems from the names frontal and temporal lobes, which are responsible for said activities. In particular, the frontal lobe is responsible for your movement, decision-making, reasoning, speaking abilities, self-control and social skills. On the other hand, the temporal lobe handles activities related to your memory, emotional expression, hearing and understanding of speech.

FTD is a progressive brain disease, meaning the parts of your brain deteriorate as time passes. Parts of the two lobes shrink, called atrophy, affecting their normal function. It is also usually defined as the early onset of dementia, although a quarter of patients show symptoms past the age of 65. This disease is a permanent, lifelong condition, giving a person an average life expectancy of 7.5 years after symptoms begin.

Are There Stages of Frontotemporal Dementia?

Unlike Alzheimer’s disease, there are no numbered stages set for FTD. The disease can affect each person differently, depending on which parts of the lobes are first affected. The gravity of the symptoms can shed light on how much the disease has affected a person.

How Does Frontotemporal Dementia Begin?

Frontotemporal dementia symptoms vary per person. Two people can have similar symptoms but may have different sets of combinations. Some will also be more severe than others. A clinical diagnosis of FTD often involves symptoms from several core domains, which include:

• Disinhibition
• Apathy
• Loss of empathy
• Impaired executive functions
• Changes in eating behavior
• Stereotyped attitude

1. Disinhibition

Disinhibition or the loss of inhibition pertains to a person’s tendency to perform inappropriate behaviors. For instance, a person with FTD may:

• Use offensive language.
• Show sexually explicit attitudes.
• Perform impulsive behaviors.
• Have a childish attitude.
• Show aggression toward specific groups or persons.
• Violate other people’s personal space.
• Easily share their personal information with strangers.

Some persons with FTD may also engage in illegal behaviors and substance abuse, such as excessively consuming alcohol and tobacco or gambling and overspending. These changes may seem like a huge personality shift for some people, making the symptom easier to spot. 

2. Apathy

Apathy, or a lack of interest, is another symptom typical of the early stages of the disease. A person with FTD may refuse to participate in social and nonsocial activities and become uninterested in goal-directed activities. 

This symptom can be hard to distinguish from having major depression. However, with apathy, the loss of interest is sudden, regardless of the person’s mood. People with FTD are also not generally suicidal or experience ideation. They typically show reduced motor activities instead. There are three subtypes to keep in mind:

• Cognitive apathy: Pertains to the reduction in voluntary and planning activities
• Behavioral apathy: Manifests in a reduced number of self-activating thoughts and behaviors
• Affective-emotional apathy: Refers to the loss of interest in activities that a person previously deemed exciting or pleasurable

3. Loss of Empathy

Empathy refers to a person’s ability to read other people’s emotions, understand their state of mind and act in an appropriate manner depending on these social cues. This skill involves recognizing emotions through visual and auditory stimuli. It helps people become aware of the negative consequences of socially inappropriate behaviors.

A person with FTD may experience an impairment of social cognition, which means they may struggle to act appropriately in relation to their environment. They may struggle to understand facial expressions or distinguish crying from laughter. Because of their reduced empathy, they may also find it challenging to make sound moral judgments and solve social dilemmas. That said, the impairment particularly centers on the person’s inability to distinguish negative emotions. 

4. Impaired Executive Functions

A person with FTD may experience executive dysfunction, which means they can:

• Get easily distracted.
• Have an impaired working memory.
• Struggle to plan or exercise their problem-solving skills.
• Have difficulty speaking or generating words.

People with FTD may belong in one of the subtypes of Primary Progressive Aphasia (PPA), a degenerative brain disease that causes you to lose your ability to speak. These subtypes include:

• Nonfluent variant: Pertains to the problems with grammar and word formation, where complicated sentences can cause the person with FTD some confusion.
• Semantic variant: Pertains to problems with choosing and understanding words, where affected persons may say phrases that don’t make sense or that they struggle to understand the words other people are saying to them.

An affected person may also experience anosognosia, or a lack of insight. This makes it challenging for them to process symptoms and understand that they might have a medical condition. With an impaired executive function, they can also experience a lack of judgment and may have difficulty following through with what they’re told to do.

Some diagnosed patients have also experienced psychosis, delusions, hallucinations and Diogenes syndrome, which includes the loss of interest in caring for one’s physical hygiene. These symptoms can be more present in the later stages of the disease.

5. Changes in Eating Behavior

A person with FTD may experience hyperorality, or the tendency to explore objects in their surroundings orally. This behavior may include smoking or compulsively putting things in their mouth, similar to what babies do. In the advanced stage of the disease, they may even ingest nonedible objects.

Their eating habits also change, where they may start to binge eat and have an increased preference for sweets. This increases the risk factors related to weight gain, like stroke, heart attack or diabetes.

6. Stereotyped Attitude

Stereotyped attitude or behavior refers to actions that are repeated over and over again, seemingly without purpose. This may involve physical actions or spoken language. For instance, they may look like tapping, lip smacking, picking or rubbing. However, they can also be a more complex set of actions. Hyperreligiosity is a rare and extreme example of this behavior, which can include heightened focus on religious practices and religious hallucinations, despite being previously religiously indifferent.

Apart from FTD, stereotypic behaviors can also be seen in other neurological and psychiatric disorders, like autism, schizophrenia and Parkinson’s disease.

At What Age Does Frontotemporal Dementia Occur?

People with frontotemporal dementia mostly develop the condition between the ages of 45 and 65. The mean age is 58. That said, only 15-22 persons out of every 100,000 are expected to have FTD. This type of dementia happens earlier compared to other age-related conditions affecting the brain, such as Alzheimer’s disease. However, it is also typically the cause of dementia later in life.

Who Is Most Likely to Get Frontotemporal Dementia?

Genetics plays a significant role in the development of frontotemporal dementia. Forty percent of the cases are hereditary. That said, DNA mutations that cause the disease can also happen at random. So, you may still develop FTD even if your parents didn’t have it. The behavioral and semantic variants are more common in men, while the nonfluent variant is more common in women.

What Causes Frontotemporal Dementia?

There is currently no known cause of frontotemporal dementia. However, understanding why it happens may help you avoid potential risk factors. 

The body’s DNA functions as an instruction manual for the cells, telling them how to do their job. With DNA mutations, the body malfunctions in how it creates proteins, producing shapes that cells can’t use or break down. If the cells can’t break down these proteins, they can’t be removed. That means they get clumped together, damaging the neurons surrounding them over time. 

These misfolded proteins also play a part in other brain conditions like Alzheimer’s disease. However, researchers have also found FTD genes in people with amyotrophic lateral sclerosis (ALS). They are still studying the connection between the two conditions.

Apart from genetics, the known risk factors currently include a history of head trauma and thyroid disease. 

How Is It Diagnosed?

A neurologist or health care provider usually diagnoses frontotemporal dementia. You may undergo a physical and neurological examination, which includes lab tests and imaging tests to review the areas of the brain that are possibly deteriorating. Tests can include:

• Magnetic resonance imaging (MRI) scans.
• Computed tomography (CT) scans.
• Positron emission tomography (PET) scan.
• Lumbar puncture or spinal tap.
• Electroencephalography.
• Blood tests.

You’ll also be asked for your medical history and undergo a neurocognitive assessment. Depending on your performance on the test, the doctor can identify whether you may have problems in certain parts of your brain.

How Is It Treated?

There is currently no known cure for FTD. That said, health care providers may recommend treatment options for the symptoms, which vary depending on each person’s case. There may also be opportunities to participate in clinical trials for experimental treatments. Keep in mind that FTD can’t be diagnosed and managed on your own. It’s best to talk with your health care provider when seeking treatment.

How to Prevent Frontotemporal Dementia

Because frontotemporal dementia happens unpredictably, there’s currently no known means to prevent it. Some studies suggest that lifestyle changes can help, even for people with family members who have a history of FTD. Being physically and mentally active seems to delay the onset of the disease or slow down its progression. According to a study comparing 105 mutation carriers and 69 noncarriers, the top 25% active carriers have experienced a functional decline that’s 55% slower compared to the least active 5% participants.

Additionally, avoiding the risks associated with FTD can be helpful, for instance, avoiding the possibility of head trauma. Drinking less alcohol, avoiding smoking and staying mentally and socially active are also known to help reduce the risk of dementia — important to note, considering FTD is an onset of this disease.

What You Can Do if Your Loved One Shows Signs of Frontotemporal Dementia

If you notice that your loved one may be experiencing symptoms of frontotemporal dementia, you may need to encourage them to talk to a health care provider. Because FTD symptoms include a lack of insight, they might not be able to recognize the symptoms on their own. If they don’t see the problem, they might not seek treatment themselves.

Additionally, people with FTD likely need a 24/7 level of care. Whether you would perform this care yourself or hire a family caregiver, it’s best to plan for their long-term needs. If you will be the one to care for them, try your best to avoid taking things personally. FTD symptoms prohibit them from controlling their behaviors and what they say, which means they may end up offending you or the people around them.

Also, you may need to ask your loved one about their specific wishes and decisions in writing. They would need someone they can trust to make the decisions for them when they won’t be able to do it themselves. These wishes may also include legal documents, which would likely need the help of an attorney.

In Summary

Frontotemporal dementia is a progressive brain disease that affects the frontal and temporal lobes. Symptoms vary per person as the disease affects each brain differently. However, for a person to be clinically diagnosed, they often have symptoms from several core domains, which include disinhibition, apathy, loss of empathy, impaired executive function, changes in eating behavior and a stereotyped attitude. 

The cause of FTD is currently unknown. However, the effects are due to the misfolded proteins that accumulate around neurons in the brain. There is currently no known treatment, but studies show that an active lifestyle may slow down its progression.