What Is Frontotemporal Dementia?
Frontotemporal dementia (FTD) is a group of diseases that damage the frontal and temporal lobes of the brain. These lobes, located on the front and sides of your brain, are important for personality, behavior, and language, so FTD can cause symptoms beyond the loss of memory and thinking abilities you might associate with dementia. It can change how a person behaves, how they use and understand language, and how they move.
FTD isn't as well known as Alzheimer's disease, the most common form of dementia. But an estimated 10%-20% of people with dementia have some form of FTD. It can have a big impact on lives, partly because it tends to strike earlier than other forms of dementia, often between ages 45 and 60.
Early symptoms, such as impulsive, rude, or odd behavior, can look like mental health problems. That's one reason FTD is often misdiagnosed at first.
Note: your doctor may call FTD frontotemporal lobar degeneration or use the term Pick's disease to refer to some cases of FTD.
Frontotemporal Dementia Types
Most cases of FTD fall into two main groups, depending on initial symptoms:
Frontal variant. This type shows up as changes in personality and behavior. It's the most common type.
Primary progressive aphasia. This type affects communication and has two subtypes:
- Progressive nonfluent aphasia, which affects the ability to speak.
- Semantic dementia, which affects the ability to understand words and recognize objects and faces.
Less commonly, people develop forms of FTD involving movement. These types include:
- FTD-ALS. About 10%-15% of people with FTD also have amyotrophic lateral sclerosis (ALS). ALS affects nerve cells in the brain and spinal cord that control muscles and the ability to speak, eat, move, and breathe.
- Parkinsonian-like FTD syndromes. Sometimes, FTD shows up as a movement disorder resembling Parkinson’s disease. One type is called progressive supranuclear palsy (PSP); another is called corticobasal degeneration.
Frontotemporal Dementia Symptoms
The symptoms of FTD differ from person to person and get worse over time. Certain symptoms tend to cluster together, depending on the type of FTD you have. But you can have more than one cluster of symptoms. They can fall into these main categories:
Behavior symptoms:
- Loss of inhibition, leading to socially inappropriate behavior, such as saying rude things or getting too close to people
- Loss of empathy, meaning you don't read the feelings of others and seem to act in cold, unfeeling way
- Apathy, meaning you don't care about things that used to be important to you
- Compulsive acts such as repeatedly clapping your hands or smacking your lips, repeating words or sounds, or watching the same movies over and over
- A decline in personal hygiene
- Changes in eating habits, which might include overeating sweets or trying to eat nonfood objects
Speech and language symptoms
- Trouble using and understanding written and spoken language
- Struggling to find the right word when speaking
- Losing the meaning of the words
- Using simpler, shorter, sentences
- Saying things that don't make sense
Movement changes
- Trouble moving the eyes
- Tremors, spasms, or twitches
- Stiffness
- Poor coordination
- Trouble swallowing
- Slurred speech
- Muscle weakness
- Inappropriate laughing or crying
- Falling or trouble walking
FTD posture
People with PSP (a type of Parkinson's-like FTD) have a typical way of standing that is different from the posture usually seen in people with Parkinson's. People with Parkinson's typically lean forward, while those with PSP typically stand upright or with an arched back. Their unstable, upright posture, possibly combined with eye movement problems, may help explain why people with PSP tend to fall backward, onto their heads.
What are the symptoms of the later stages of FTD?
As FTD progresses, symptoms get worse, and people eventually need intense care. People who had only behavior, speech, or movement problems at first can develop problems in the other areas. The differences between the types of FTD tend to blur over time. Memory problems that were absent at first tend to become more prominent. But the pace and pattern of change varies from person to person.
Frontotemporal Dementia Causes and Risk Factors
If you have FTD, parts of your brain shrink as cells called neurons die off. Scientists believe that certain proteins made by your body play a role in this damage. Abnormal (misshapen and malfunctioning) forms of these proteins, called tau and TDP-43, build up in the damaged brain regions.
About 40% of people with FTD have a family history of the disease. In some cases, FTD has been linked to specific genetic mutations that run in families.
Genes that can cause FTD include:
- Tau gene (also called the MAPT gene), linked most often to the behavioral type of FTD
- GRN gene, also mostly linked to behavioral FTD
- C9orf72 gene, linked to both FTD and ALS
Other factors may raise your risk of developing FTD:
- A history of head injury, which more than triples your risk
- Thyroid disease, which increases your risk 2.5 times
Frontotemporal Dementia Diagnosis
It can be hard to figure out if someone has FTD. That’s because it often mirrors other disorders, such as Parkinson's, Alzheimer's, depression, and schizophrenia.
See a doctor about any strange behavior. They’ll ask about medications and family history. They may also do blood tests to rule out other medical problems. Your doctor might refer you to a neurologist, who will check things such as your balance, reflexes, memory, and thinking.
Frontotemporal dementia tests
There's no single test for FTD. But to confirm a diagnosis in someone with suspicious symptoms, a neurologist might order tests including:
- CT scan. It shows a detailed image of your brain, made with X-rays.
- MRI. This uses magnets and radio waves to create images of your brain.
- Fluorodeoxyglucose positron emission tracer (FDG-PET) scan.A low-level radioactive substance injected in your blood shows up in the brain, revealing how different parts are working.
- EEG (electroencephalogram). This measures the electrical activity in your brain and can detect seizures.
- Spinal tap. This samples fluid around your brain and spinal cord.
- Sleep study. This is a way to rule out a sleep disorder, such as sleep apnea, that might be causing your symptoms. You might spend a night in a clinic or use equipment at home to monitor your sleep.
- Neuropsychological testing. You might fill out questionnaires and take tests involving writing, drawing, solving puzzles, and other tasks. This allows a specially trained psychologist to look at your mental abilities and patterns, including your skills in memory, language, reasoning, planning, and attention.
Frontotemporal Dementia Treatment
FTD doesn’t have a cure or treatment. Drugs that slow down Alzheimer’s disease don’t have the same effect on FTD and may even make symptoms worse.
The doctor can help you manage your symptoms with certain medications and therapies, including:
- Antidepressants to help with mood and behavior
- Antipsychotics to help with behavioral issues, though they may cause serious side effects, such as a higher risk of death
- Speech therapy to help with communication problems
Living With Frontotemporal Dementia
If you or a family member are diagnosed with FTD, it's important to find a doctor who knows how to manage it as soon as possible. Speech experts, physical therapists, and nurses may be able to help, too.
Keep in mind that if you have FTD, you will lose the ability to understand and manage the condition yourself. You will likely lose control of your behavior, your ability to speak, and your ability to understand others.
If you are still able, you should consider:
- Asking your doctor whether medications can help
- Finding a support group
- Sharing information with family and friends
- Getting a driving evaluation
- Exercising and eating healthy foods
- Creating a plan for when your ability to live on your own and make decisions is impaired. You'll need someone to take over all your responsibilities, including your finances, at some point.
- Making safety modifications to your home, such as removing rugs that are tripping hazards and installing raised toilet seats
If you’re caring for someone with FTD, you can:
- Talk with professionals about how to interact with your loved one
- Keep a log of behavioral symptoms to identify triggers and skip events or activities that lead to problems
- Provide structured daily routines
- Simplify daily tasks
- Explain to others what to expect when they’re with your loved one
- Connect with others through caregiver support groups
- Find respite caregivers who can step in when you need a break
- Take care of your own physical and mental health by eating well, getting enough sleep, exercising, and spending time doing things you like to do
- Make plans for when your loved one will need round-the-clock care and supervision. Many people with FTD eventually need nursing home care.
Frontotemporal dementia life expectancy
The average life expectancy following an FTD diagnosis is 7.5 years. The disease isn't fatal on its own, but as brain damage progresses, it causes problems that can be dangerous or life-threatening. For example, swallowing problems increase the risk of pneumonia, which is the leading cause of death. Fall-related injuries and deaths also are common.
Takeaways
Frontotemporal dementia is a group of conditions that usually start with changes in behavior, language, or movement, and eventually rob people of the ability to communicate or live without constant care and supervision. There's no cure, but there are things you and your loved ones can do to cope.
Frontotemporal Dementia FAQs
How does frontotemporal dementia start?
This can vary a lot, but the most common early symptoms involve behavior. You might notice changes in personality or mood or a new lack of social skills. The first sign might be poor hygiene or weight gain from binge eating. The person might not notice or care about the changes.
What are the 7 stages of frontotemporal dementia?
While some health care providers have made up lists of typical FTD stages, outlining seven or eight phases, most experts say there's no single predictable pattern. For one thing, FTD comes in several different types. The most common types start with behavioral or communication problems, but other types start with movement issues. People with any type get worse over time and often develop symptoms in all three areas. But the pace and pattern of change differ from person to person.
How long does end-stage frontotemporal dementia last?
It's hard to predict. Late in the disease, someone with FTD will need intense supervision and care, often in a nursing home. But how long they live in that state depends on many factors, including their age when they were diagnosed and whether they have other health problems.
