Frontotemporal Dementia

Frontotemporal dementia (FTD) is a group of brain disorders caused by atrophy of the frontal and temporal lobes. The condition is not treatable, so associated symptoms – significant behavioral changes, motor disorders, and loss of language skills – persist and worsen with time.

What is frontotemporal dementia?

FTD is a cognitive disorder that affects a person’s behavior, motor abilities, and language skills. These changes result from shrinking frontal and temporal lobes, the areas of the brain that control behavior, movement, and language.

The clinical symptoms associated with FTD depend on the lobe that’s affected. If shrinkage is in the frontal lobes, personality and motor control changes will result. If shrinkage is in the temporal lobes, a loss of language comprehension is more likely to occur. Some people experience shrinkage in both lobes, resulting in several clusters of symptoms occurring simultaneously.

FTD is often misdiagnosed as Alzheimer’s disease or a psychiatric condition, depending on the prevalent symptoms. However, FTD typically presents much earlier than dementia and is the most common form of dementia for people 60 years old and younger.^[1]

Types of frontotemporal dementia

FTD was known initially as Pick’s disease. However, as research shed light on this condition and related neurodegenerative diseases, Pick’s disease was grouped with semantic dementia and primary progressive aphasia under the umbrella term frontotemporal dementia.^[2] 

As a result of this grouping, researchers have identified three distinct types of frontotemporal degeneration, each of which has very different symptoms:

• Behavioral variant – Known as bvFTD, this version involves a loss of control regarding behavior. Symptoms include inappropriate social behavior, a loss of ability to recognize others’ feelings, and reduced language and cognitive skills. People with bvFTD are typically unaware of their behavioral changes.^[3]
• Primary progressive aphasia – Known as PPA, this type of FTD degeneration focuses on the loss of language abilities in both the receptive and expressive realms. PPA most often involves the inability to name objects and find words, though other symptoms, including motor speech problems and reduced language comprehension, are often present.^[4]
• Progressive nonfluent aphasia – Known as nfvPPA, this type of FTD dementia affects one’s ability to produce speech, which becomes increasingly halting. Speech might become slurred and slower with time as the patient struggles to get words out.^[5]

Symptoms of frontotemporal dementia

As the frontal and temporal lobes in the brain shrink due to FTD, the functions associated with those areas of the brain begin to deteriorate.

The frontal lobe controls personality and movement, among other things, while the temporal lobe controls language, perception, and memory, to name a few. As neurons in these areas stop working, the primary FTD symptoms discussed below begin to appear. With time, these symptoms become more severe.

Behavioral changes vary widely but commonly include:^[3]

• Compulsive behaviors include repetitively tapping fingers, clapping, or repeating the same word or phrase.
• Mouth-based behaviors include overeating, eating things that are not food (known as Pica), or compulsive behaviors like smoking.
• Personality changes, such as a loss of inhibition, loss of empathy, and an increase in apathy, which often presents as a loss of motivation or decline in personal hygiene.
• Inappropriate social behaviors, including temper tantrums, touching strangers, and public urination.
• Loss of executive functioning, such as a deterioration of problem-solving and organizational abilities.

Other common behavioral symptoms include impulsivity, social withdrawal, and feelings of restlessness. An increased interest in sex, agitation, or blunted emotions may also occur. In many cases, the patient is unaware that their behavior has changed, let alone that it might be inappropriate.

It should be noted that memory loss is a behavior change that does not occur until the later stages of the disease.

Motor control issues are less common than the behavioral changes listed above and the types of linguistic deterioration discussed below. Nonetheless, people with FTD might experience symptoms similar to those seen with Lou Gehrig’s disease:^[6]

• Involuntary muscle movements, such as tremors, twitches, or muscle spasms. Rigidity and muscle weakness are also common.
• Poor coordination, such as difficulty walking.
• Difficulty swallowing.

Loss of language skills commonly present as aphasia:^[4][5]

• Primary progressive aphasia involves trouble finding words and naming objects. For example, a person might use the word “it” to describe their dog.

Poor language comprehension, repetitive language, poor grammar, and nonfluent speech (e.g., broken and halting speech) are also common. Eventually, someone with PPA might completely lose their language skills.

• Progressive nonfluent aphasia involves difficulty with verbal expression. While patients still understand the meaning of words, the ability to pronounce words and put complex sentences together diminishes.

Improper grammar, such as omitting adjectives from sentences, is also a common symptom. Typically, people with this disorder compensate by speaking in shorter sentences.

The ability to read and write often remains adequate, so using written words to communicate is a common accommodation. As the disease progresses, difficulty expressing words might become so severe that language ceases completely. 

Causes of frontotemporal dementia

Two events cause FTD: shrinkage of the frontal and temporal lobes and a build-up of proteins in the cells of these lobes.^[7] As a result, the hallmark symptoms of FTD discussed earlier – behavioral changes, linguistic limitations, and motor control issues – start to present.

It is not understood why these events occur, however. It’s believed that genetics play a part, though it is not the only factor. Research varies regarding the heritability of FTD. Some studies suggest that one in eight people with FTD dementia have a relative with the same condition^[8], while other studies put that number at about three in eight.^[9] 

Diagnosing frontotemporal dementia

As mentioned earlier, FTD symptoms are similar to those of Alzheimer’s disease and several psychiatric disorders, which makes an accurate FTD diagnosis somewhat difficult.

While there is no specific test for FTD, various diagnostic methods are available to assess a patient’s condition. Diagnosis begins with a thorough discussion of the patient’s medical history, as well as a neurological examination. Additionally, common tests include:^[8]

• Magnetic Resonance Imaging (MRI), which aids in determining the location and level of atrophy in the brain.
• Positron Emission Tomography (PET), which shows glucose and amyloid levels in the brain. Reduced glucose levels indicate that an area of the brain is not functioning normally. Elevated amyloid levels are associated with Alzheimer’s disease, so this test helps to rule out FTD.
• Spinal tap, in which cerebrospinal fluid (CSF) is taken via a thin needle inserted into the spinal cord. Analysis of CSF can help rule out other conditions with symptoms similar to FTD, such as cancer. Likewise, analysis can also detect proteins collected by CSF as it moves around the brain.

Additionally, a neuropsychological evaluation determines a person’s functionality related to language, motor movements, memory, and executive functioning.

Prevention of frontotemporal dementia

The development and manifestation of FTD is highly unpredictable. For example, people with the same genetic mutations experience FTD in varying degrees.^[9] For this reason, identifying ways to prevent its development has been difficult.

However, research indicates that a physically and mentally stimulating lifestyle might help stave off symptoms in people with a genetic predisposition for the disease.^[9] Additionally, there might be a connection between traumatic brain injury (TBI) and the development of FTD. As such, preventing head injuries could decrease the chance of getting FTD disease.^[10]

Treatment for frontotemporal dementia

There is no treatment to prevent FTD from developing. There is also no cure. However, some of the symptoms might be relieved through medication or therapy.

Medications include:^[6]

• Antidepressants, such as Zoloft, which help minimize obsessive-compulsive behaviors and anxiety. This is the most common type of medication for FTD symptoms.
• Antipsychotics, such as Risperdal, which help minimize impulsivity, compulsive behaviors, and irrational thoughts. Antipsychotics are used less often and are typically only prescribed if antidepressants do not have the desired effect.
• Sleep aids, such as Dalmane, which minimize sleep disturbances.

Therapies include:

• Speech and language therapy, which treats reading, writing, language production, and comprehension deficits.^[11]
• Behavior modification, which addresses risky or inappropriate behaviors, improves functionality, and enhances support.^[12]
• Physical and occupational therapy, which addresses associated motor disorders, such as muscle weakness, poor coordination, and difficulty walking.^[13]

Self-care for frontotemporal dementia

If you have been diagnosed with FTD, there are many strategies you can implement to help manage your symptoms and minimize the impact of this disease on your daily life.

In addition to following your doctor’s advice regarding medications and therapies, you can:^[7]

• Try relaxation techniques, like meditation or music therapy, to help calm you down when you are anxious.
• Join a support group for people with FTD disease to share your experiences.
• Exercise and eat well. This will help you feel better physically and emotionally.
• Seek out social interaction, like participating in leisure activities with friends and family.
• Maintain a structured daily routine to help manage anxiety, maintain hygiene, and minimize impulsive behaviors.

Furthermore, it’s important to ask for and accept help from others, particularly as the disease progresses.

Helping someone with frontotemporal dementia

Helping or caring for people with this type of degeneration can be challenging, particularly if the person you’re caring for is a loved one. It can be stressful and cause you anxiety and burnout. Even if you aren’t the primary caregiver, witnessing the decline of a loved one can be highly emotionally taxing.

To provide the best assistance to people with FTD, you can:

• Be patient and supportive
• Provide respite for primary caretakers, or seek respite services if you are the primary caretaker
• Educate yourself about the disease
• Seek support services to assist the FTD patient
• Seek support for yourself as an FTD caretaker
• Encourage FTD patients to seek care, as appropriate
• Be realistic about your ability to provide care, and seek long-term care solutions when necessary

As discussed above, there is no specific treatment for FTD and no cure. Once someone develops symptoms, their condition will only worsen. But, with proper support for them and for you, you can help make the most of the time an FTD patient has left.

FAQs about frontotemporal dementia

How does frontotemporal dementia impact daily life?

FTD can have significant impacts on one’s ability to function. Aside from the common motor, linguistic, and behavioral symptoms described earlier, people with FTD can have difficulty completing tasks at work, maintaining relationships with others, and engaging in leisure activities.

Likewise, as the disease progresses, people with FTD can have trouble caring for themselves, such as with feeding and using the restroom. Other activities of daily living, such as paying bills, cleaning the house, and bathing, are often severely impacted as well.

How common is frontotemporal dementia?

Rough estimates indicate that about 10-20 percent of dementia cases are FTD. This represents about 50,000-60,000 people in the United States. Worldwide, rates of FTD are thought to be around 15-22 cases per 100,000 people.^[14] These estimates are over a decade old, however, so the prevalence of this disease today might be different.

What is the outlook for people with FTD?

FTD is an incurable, lifelong condition. While the treatments discussed earlier can help manage symptoms to a degree, the symptoms will get progressively worse.

Some people with FTD deteriorate very quickly (in less than two years). Others live much longer with milder symptoms (more than ten years). Many people with FTD eventually need 24-hour care in an institutionalized setting.^[2]

Frontotemporal dementia vs Vascular dementia – What is the difference?

These types of dementia are highly similar and can often be difficult to distinguish.^[15] However, differences in symptomatology can help identify one from the other:

• Symptoms of FTD can manifest as marked changes in behavior, reduced motor abilities, or increased language difficulties. Memory loss is often not severe until the latter stages of the disease. Onset is typically between the ages of 50-69.^[16]
• Symptoms of vascular dementia revolve around memory loss, poor planning and organization, and an inability to concentrate. Depression, aggression, delusions, and hallucinations might also occur. Onset is usually after age 65.^[17]