Understanding the Stages of Frontotemporal Dementia

Frontotemporal dementia (FTD) is a type of dementia that impacts the frontal and temporal lobes of your brain. These areas are important for many cognitive functions, including:

• personality
• decision-making
• planning or problem-solving
• self-control
• attention
• speech
• movement
• memory
• hearing
• facial and language recognition

There are two main types of FTD:

• behavioral variant FTD (bvFTD): bvFTD is the most common type of FTD. It’s predominantly associated with changes in personality, judgment, and behavior.
• primary progressive aphasia (PPA): Individuals with PPA have difficulties with language and communication. There are two subtypes of PPA:
  • semantic variant: when a person has trouble finding words and forming sentences
  • nonfluent variant: where an individual has trouble speaking and may also use incorrect grammar when they do

Some people with FTD also have a less common variant that’s associated with difficulties related to movement. When this occurs, symptoms can be similar to amyotrophic lateral sclerosis (ALS) or Parkinson’s disease.

FTD is a progressive condition. When a condition is progressive, it means that its symptoms get worse as time passes.

While there are no firmly defined stages of FTD, people with the condition typically progress through several general stages. Each of these is characterized by the appearance or worsening of certain symptoms.

In early stage FTD, there may be few symptoms, or symptoms may develop subtly. Because of this, it can be easy to initially brush off early FTD symptoms as a typical part of aging.

Generally speaking, the most prominent symptoms can depend on the specific type of FTD a person has. Some early symptoms of bvFTD include things like:

• socially inappropriate actions
• impulsive behaviors
• apathy, which is a lack of interest or enthusiasm in things like work, hobbies, or social interactions
• reduced empathy, which is a lack of concern for others and their needs
• changes in dietary habits, such as overeating or increased consumption of sweet foods or alcohol
• repetitive movements or statements

Meanwhile, some possible early symptoms of PPA may include:

• trouble understanding words
• problems finding or recalling words
• difficulty with speaking coherently
• misusing words or using incorrect grammar
• issues with understanding longer or more complex sentences

Unlike other types of dementia, many people with early stage FTD don’t have problems with memory. Because of this, it’s possible that people in this stage of FTD may be misdiagnosed with a psychiatric condition.

For example, in addition to being a symptom of bvFTD, apathy also happens in depression, which isn’t uncommon in older adults. In fact, the World Health Organization (WHO) estimates that depression affects 7% of this population.

As such, it’s possible that someone with early stage bvFTD could be misdiagnosed with depression. They may not be evaluated for FTD or other types of dementia until their condition has progressed to a later stage.

Middle stage FTD is characterized by a worsening of the symptoms that started in early stage FTD.

For example, people with bvFTD can have more frequent or serious behavioral problems, and people with PPA can experience increased communication troubles.

There may also be an overlap of symptoms. This means that a person with PPA may display an increasing number of behavioral changes, or a person with bvFTD may have rising communication problems.

In late stage FTD, memory can also start to be affected, meaning that a person will have memory symptoms commonly associated with other types of dementia, such as Alzheimer’s disease. Examples include:

• forgetting more recent events or even older events
• becoming confused or lost in familiar places, such as at home or in the neighborhood
• not being able to learn new things
• not recognizing friends and family

Further, people with late stage FTD may also have increasing difficulties doing daily activities, such as:

• eating and drinking
• bathing
• dressing
• grooming
• using the toilet

In addition to worsening cognitive decline, movement troubles also increase as someone with FTD enters the late stages of the condition. This can mean that they may need to use a wheelchair or may become bedbound.

Further, muscles can begin to weaken. While this can increase movement difficulties, it can also lead to problems with chewing and swallowing or maintaining bowel and bladder control.

As such, people with late stage FTD typically need close monitoring and care each day to ensure that necessary daily tasks are completed as well as to maintain their safety and well-being.

The exact cause of FTD is unknown. However, the condition is associated with some changes that occur in the brain. One of these is a loss of nerve cells in the frontal and temporal lobes.

Another change is an accumulation of atypical forms of two proteins called tau and TDP-43. While these proteins occur naturally, when they don’t function normally, they can damage nerve cells.

There’s also a genetic aspect to FTD. Researchers have identified several gene changes associated with FTD.

FTD can be difficult to diagnose. This is because its symptoms can be similar to other conditions, including other types of dementia.

A doctor will typically use the following to help diagnose FTD:

• a thorough personal and family medical history
• a physical exam
• an evaluation of your current symptoms
• a psychiatric assessment
• blood or CSF tests to help rule out other conditions
• tests that measure cognitive factors like thinking, memory, and language
• tests that gauge your level of physical functioning
• imaging tests like CT scan, MRI scan, or PET scan
• genetic testing (if a genetic cause is suspected)

FTD does not have a cure. At this time, there are also no effective ways to stop the progression of the condition. Because of this, treatment focuses on managing symptoms and improving quality of life.

The treatment of FTD typically involves a multidisciplinary care team. The specific interventions that may be recommended can depend on the types of symptoms that a person has, but may include:

• medications to help improve behavioral symptoms
• physical therapy
• occupational therapy
• speech and language therapy
• behavioral modification
• lifestyle and environmental changes
• assistance with carrying out daily tasks

FTD gets worse over time. However, the rate at which someone progresses through the stages of FTD will vary greatly between individuals.

Additionally, the fact that FTD often has an early and subtle onset can lead to delays in diagnosis. This means that many people with FTD may not receive the care that they need until they’ve progressed to later stages of the condition.

Indeed, a 2021 study found that, compared to Alzheimer’s disease, people with FTD had a longer time until referral to a specialist and longer delays in diagnosis.

The average survival time for FTD is 7.5 years. However, research has found that people with FTD with motor disorders have a shorter survival time than those with bvFTD or PPA.

Now let’s look at a few more questions that you may have about FTD.

How common is frontotemporal dementia?

FTD is rather uncommon compared to other types of dementia. It’s estimated to affect 15 to 22 per 100,000 people.

What’s the most common type of dementia?

Alzheimer’s disease is the most common type of dementia. The WHO estimates that it accounts for 60% to 70% of dementia diagnoses.

When is frontotemporal dementia typically diagnosed?

Compared to other types of dementia, FTD is more often diagnosed at a younger age. According to the National Institute on Aging, about 60% of people who’ve been diagnosed with FTD are between the ages of 45 and 64.

Who is at risk for frontotemporal dementia?

People with a family history of FTD are at an increased risk. It’s estimated that 30% of people with FTD have a strong family history of the condition. BvFTD is more strongly associated with heritability than other types of FTD.

Can frontotemporal dementia be prevented?

There’s no known way to prevent the onset of FTD. However, a 2020 study did find that increasing mental and physical activity levels could slow cognitive decline in people who had inherited forms of FTD.

FTD is a type of dementia that affects the frontal and temporal lobes of the brain. It can lead to progressive worsening of changes in personality, behavior, and communication.

There are three general stages of FTD. The symptoms of early stage FTD come on slowly and depend on the type of FTD a person has.

In middle stage FTD, symptoms worsen and may be symptom overlap between the main two types of FTD. In late stage FTD, symptoms begin to resemble other more common types of dementia like Alzheimer’s disease.

There’s no cure for FTD. Treatment works to manage symptoms and boost quality of life. If you notice that a loved one is showing new or worsening symptoms related to behavior or communication, consider consulting a doctor for an evaluation.