Understanding and coping with the stages of frontotemporal dementia

If you or someone you love has been diagnosed with frontotemporal dementia (FTD), it’s understandable to feel upset, overwhelmed, or unsure about what comes next. FTD is a life-altering disease that affects between 50,000 and 60,000 Americans, as well as their family members and friends. Any dementia diagnosis means accepting that the future may look different than you planned, which can be difficult to process. Learning what to expect can help you begin planning. Here’s what you need to know about FTD.

The 7 stages of frontotemporal dementia: what to know

Let’s start by clearing up a common misconception. Frontotemporal dementia doesn’t have 7 distinct stages. Instead, this degenerative disease is generally described in 3 broad stages: early, middle, and late.

The confusion about 7 stages of frontotemporal dementia likely comes from applying another dementia staging system called the Global Deterioration Scale (GDS). The well-known Global Deterioration Scale, also known as the Reisberg Scale, consists of seven stages of cognitive decline. It’s designed to evaluate the progression of Alzheimer’s disease, which is the most common form of dementia.

However, FTD differs from Alzheimer’s disease in both its symptoms and progression. Although the Reisberg Scale offers a general framework for understanding the broad progression of dementia, it doesn’t accurately capture how the specific symptoms of FTD develop.

What is frontotemporal dementia (FTD)?

Frontotemporal dementia is one of several types of dementia. FTD primarily affects the brain’s frontal and temporal lobes, which are crucial for cognitive, behavioral, and language functions.

The role of the frontal and temporal lobes

The frontal lobes control executive functions, including problem-solving, planning, organizing, impulse control, and social behavior. The temporal lobes, meanwhile, play an important role in memory and language processing. The symptoms a person experiences can reflect which of these areas is affected.

3 subtypes of frontotemporal dementia

FTD has three common subtypes, and each is associated with a general group of symptoms:

• Behavioral variant (bv-FTD): This subtype causes significant changes in personality and social behavior, such as disinhibition, apathy, emotional blunting, declining personal hygiene, or compulsive behaviors. These changes can disrupt personal and professional relationships. It’s also often called Pick’s disease.

• Semantic dementia (SD): This subtype involves a progressive loss of the ability to understand and use language. A person may have difficulty recognizing words and objects, eventually leading to a severe breakdown in their ability to communicate.

• Progressive nonfluent aphasia (PNFA): This subtype causes difficulty producing speech, including halting or effortful speech, grammatical errors, and problems with pronunciation. Comprehension and nonverbal cognitive skills remain relatively preserved.

How is frontotemporal dementia diagnosed?

Only a licensed medical doctor, most often a neurologist, can diagnose FTD. Your doctor will usually perform a series of tests to determine what’s causing the symptoms. Documenting early symptoms, including when you first noticed them, can help support the diagnostic process. Knowing what to expect during appointments may also help you feel more prepared. Don’t hesitate to ask the doctor questions about the process.

With that in mind, here’s what you can generally expect on the path to an FTD diagnosis.

• Neurological examination

• Neuropsychological testing

• Neuroimaging

• Assessment of medical history

• Blood tests and a lumbar puncture, also called a spinal tap, if needed to rule out other conditions

Frontotemporal dementia: what to do after a diagnosis

Receiving a frontotemporal dementia diagnosis is life-altering for the person diagnosed, their family members, and their friends. Although there is no cure for the disease itself, planning for the road ahead can make a meaningful difference in how everyone experiences the journey.

Here are six important steps to consider after an FTD diagnosis:

• Learn about FTD: Understanding FTD’s different subtypes, symptoms, and progression can help you manage the condition more effectively. Knowledge can also help you and your family prepare for what may lie ahead and recognize how needs might change.

• Choose your care team: Work with a neurologist, speech therapist, occupational therapist, and other health care professionals who can help manage the different symptoms and challenges associated with FTD. Take time to interview different providers so you can build a team that listens to and respects your perspectives. A strong support team can help address evolving medical needs.

• Plan for the future: Because FTD is progressive, it’s important to begin financial and legal planning early. Talk with your family and a trusted legal advisor about establishing power of attorney, creating advance directives, and addressing estate planning. Starting these discussions proactively can help everyone understand the plans.

• Consider your care options: Because FTD is progressive, it can become difficult to manage in a home setting. Memory care communities have professionally trained staff with experience supporting people living with dementia. Take time to research and tour memory care communities. Work with your family members and care team to decide when a move might be appropriate based on changing needs.

• Join support groups: Connecting with other people affected by FTD, including people diagnosed with the condition and family caregivers, can provide valuable emotional support and practical advice. Local or online support groups offer a place to share experiences, learn from other people’s journeys, and find comfort in knowing that you’re not alone.

• Prioritize self-care and wellness: Living with FTD can be challenging, making physical and emotional well-being especially important. Focus on maintaining a healthy lifestyle through regular exercise, balanced nutrition, and stress management techniques, such as meditation or counseling, to support overall quality of life.

The 3 stages of frontotemporal dementia: what to expect

Every person’s symptoms and experience will differ based on the type of FTD they have and their individual traits. According to the Association for Frontotemporal Association, a common saying among people experiencing FTD is, “If you’ve seen one case of FTD…you’ve seen one case of FTD.”

Although the later stages of disease progression look different for everyone, a person with FTD will generally experience three stages. Each stage brings a worsening of symptoms from the previous stage. It’s important to consider potential long-term care needs at every stage so you’ll be better prepared if that time comes.

Early-stage frontotemporal dementia

During early-stage FTD, a person may not have received a diagnosis yet or may have been misdiagnosed with another condition. Symptoms are often subtle during this early stage, which can make them difficult to recognize. Unlike some other types of dementia, people with early-stage FTD often show no signs of memory loss.

Early-stage symptoms of frontotemporal dementia

Depending on the type of FTD, you may notice some or all of the following behavioral, communication, or movement-related symptoms.

Behavioral symptoms (bvFTD)

• Selfish or unempathetic behavior

• Socially inappropriate behavior

• Apathy or decreased interest in usual activities

• Repetition of words, movements, or behaviors

• Impulsive or reckless behavior

• Aggression

Language and communication symptoms (PPA)

• Difficulty forming sentences

• Difficulty understanding sentences

• Difficulty speaking clearly

Motor symptoms (ALS with FTD, CBS, PSP)

• Muscle stiffness

• Slow movement

• Poor balance and more frequent falls

• Difficulty completing usual tasks

Behavioral symptoms of early-stage FTD

The following symptoms may occur with behavioral variant FTD:

• An increase in selfish or unempathetic behavior

• Socially inappropriate behavior

• Apathy or decreased interest in usual activities

• Unusual repetition of words, movements, or behaviors

• Impulsive or reckless behavior

• Aggression

Language-related symptoms of early-stage FTD

The following language and communication symptoms typically occur with primary progressive aphasia:

• Difficulty forming sentences

• Difficulty understanding sentences

• Difficulty speaking or being understood by others

Motor symptoms of early-stage FTD

The following symptoms may occur with FTD subtypes that affect movement:

• Muscle stiffness

• Slow movement

• Poor balance and more frequent falls

• Difficulty completing usual tasks

Middle-stage frontotemporal dementia

During middle-stage FTD, symptoms from the early stage progress or become more severe. If a person experienced behavioral symptoms during early-stage FTD, they may notice more impulsive or reckless behavior. If they experienced PPA during the early stage, their language difficulties may become more pronounced.

A person may also begin experiencing symptoms associated with other variants. For example, someone who initially experienced behavioral symptoms may develop both behavioral and language-related changes during middle-stage FTD.

People with middle-stage frontotemporal dementia are also likely to begin having difficulty with activities of daily living (ADLs), such as getting dressed and bathing. These changes may mean that the person needs more support with their usual routines.

Late-stage frontotemporal dementia

During late-stage FTD, symptoms continue to worsen. Activities of daily living that became difficult during the middle stage may now be increasingly difficult and require more support. A physician may prescribe antidepressants or antipsychotic medications to help make symptoms more manageable.

Another sign of late-stage frontotemporal dementia is memory loss. This may include confusion, forgetting things that recently occurred, and not recognizing loved ones. If you haven’t already, this may be a time to consider memory care options that can provide specialized support for the person’s changing needs.

Help for frontotemporal dementia family caregivers

Caring for someone with FTD can be emotionally and physically challenging, especially as you work to ensure they receive quality care. Knowing which resources fit your needs and budget can provide valuable support throughout your caregiving journey.

Understanding the stages of frontotemporal dementia can help both people living with FTD and the family members and friends who support them prepare for changing needs.

Throughout this journey, remember that people living with frontotemporal dementia aren’t defined by their condition. By understanding their evolving needs, learning about the stages of FTD, seeking professional guidance, and offering steady support, you can help them navigate the challenges of daily life with dignity.