Understanding and Coping with the Stages of Frontotemporal Dementia

If you or someone you love has been diagnosed with frontotemporal dementia (FTD), feeling upset and lost about how to move forward is expected. FTD is a life-altering disease that impacts between 50,000 and 60,000 Americans, and it impacts patients and family members alike. A dementia diagnosis of any type means accepting that the future may look different than you planned, which can feel overwhelming. Here's what you need to know about FTD:

The 7 stages of frontotemporal dementia: what to know

First, let's dispel a widespread myth. Contrary to a common misconception, there are not 7 distinct stages of frontotemporal dementia (FTD), but rather 3 broad stages of this degenerative disease.

The confusion about there being 7 stages of frontotemporal dementia (FTD) likely arises from a misapplication of 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 and is designed to evaluate the progression of Alzheimer's disease, which is the most common form of dementia.

However, FTD is different from Alzheimer's disease and has a different pattern of symptoms and progression. While the Reisberg Scale provides a general framework for understanding the broad progression of dementia, it does not accurately capture the specific symptom development in FTD.

What is frontotemporal dementia (FTD)?

Frontotemporal dementia is one of several types of dementia. FTD primarily impacts the frontal and temporal lobes of the brain, regions that are crucial for various cognitive, behavioral, and linguistic functions.

The role of frontal and temporal lobes

The frontal lobes are responsible for controlling executive functions, including problem-solving, planning, organizing, impulse control, and social behavior. The temporal lobes, on the other hand, play a vital role in managing memory and language processing.

3 subtypes of frontotemporal dementia

There are three common subtypes of FTD, and each subtype is associated with a general collection of symptoms:

• Behavioral variant (bv-FTD): Is characterized by significant changes in personality and social conduct, such as disinhibition, apathy, emotional blunting, and a decline in personal hygiene or even compulsive behaviors, often leading to disruptions in personal and professional relationships. This is also often called Pick's Disease.
• Semantic dementia (SD): Involves the progressive loss of understanding and use of language, with patients experiencing difficulty in recognizing words and objects, eventually leading to a severe breakdown in communication abilities.
• Progressive nonfluent aphasia (PNFA): Is marked by deficits in speech production, marked by halting and effortful speech, grammatical errors, and difficulties in pronunciation, while comprehension and non-verbal cognitive skills are relatively preserved.

How are frontotemporal dementias diagnosed?

Only a licensed medical doctor, most often a neurologist, can diagnose FTD. While your doctor will often do a series of tests to determine the cause of symptoms, it's helpful to document early symptoms to support the diagnostic process. Knowing what to expect at the appointments will help you be more prepared. And remember, don’t hesitate to ask the doctor questions.

With this in mind, here is generally what you can expect on the path to an FTD diagnosis.

• Neurological examination
• Neuropsychological testing
• Neuroimaging 
• Assessment of medical history
• Blood tests and lumbar puncture (spinal tap), if needed, to rule out other conditions

Frontotemporal dementia: what to do after the diagnosis

Receiving a diagnosis of Frontotemporal dementia (FTD) is a life-altering event - for the patient, their family members and friends. And while there is no cure for the disease itself, planning for the journey ahead can make a big difference in how everyone experiences the journey.

Here are six essential steps to consider following an FTD diagnosis:

• Learn about FTD: FTD has various subtypes, symptoms, and progression to better understand the disease and manage your condition effectively. Knowledge is power, and understanding FTD will help you and your family prepare for what lies ahead.
• Pick your team: Collaborate with a neurologist, speech therapist, occupational therapist, and other healthcare professionals who can help you manage the diverse symptoms and challenges associated with FTD. Take the time to interview different providers to ensure that you have a team that listens and respects your viewpoints. Having a strong support team in place is crucial for addressing your evolving medical needs.
• Plan for the future: Considering the progressive nature of FTD, it is essential to proactively engage in financial and legal planning. Discuss with your family and a trusted legal advisor to establish a power of attorney, set up advance directives, and address estate planning.
• Consider your care options: As a progressive disease, FTD can be hard to manage in a home setting. Memory care communities have professionally trained staff who are expert in working with dementia patients. Take the time to research - and even tour - memory care communities. Work with your family members and care team to decide when the right time for a move might be.
• Join support groups - Connecting with others who have been affected by FTD, either as patients or caregivers can provide valuable emotional support and practical advice. Engage with local or online support groups, where you can share experiences, learn from others' journeys, and find comfort in knowing you are not alone.
• Emphasize self-care and wellness - Living with FTD is a challenging experience, making it even more vital to prioritize physical and emotional well-being. Focus on maintaining a healthy lifestyle through regular exercise, balanced nutrition, and stress management techniques, such as meditation or counseling, to improve overall quality of life.

The 3 stages of frontotemporal dementia: what to expect

Every patient's symptoms and experience will be different on the type of FTD they have and also based on their own unique 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.” 

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

Early-stage frontotemporal dementia

In early-stage FTD, you may not have a diagnosis yet or have even been misdiagnosed with another condition. This is because it is common for symptoms to be subtle in the early stages of the disease. And, unlike some other types of dementia, people with early-stage FTD often show no signs of memory loss. 

A summary: early-stage symptoms of frontotemporal dementia

Depending on the type of FTD, you may notice some or all of these symptoms. 

 Behavioral symptoms of early-stage FTD

These symptoms may be present in the behavioral variant FTD:

• An increase in selfish and unempathetic behaviors
• Social inappropriateness
• Apathy, or a decrease of interest in usual activities
• Unusual repetition of words, movements, or behaviors
• Impulsiveness or reckless behavior
• Aggression

Language-related symptoms of early-stage FTD

These symptoms are typically present in primary progressive aphasia:

• Difficulty forming sentences
• Difficulty understanding sentences
• Trouble speaking or speaking understandably to others

Motor symptoms of early-stage FTD

These symptoms may be present in FTD subtypes that affect movement: 

• Muscle stiffness
• Slow movement
• Poor balance and more frequent falling
• Difficulty completing usual tasks

Middle-stage frontotemporal dementia

Middle-stage FTD is characterized by a progression or worsening of the symptoms experienced in the early stage. If you experienced behavioral symptoms in early FTD, you should expect those symptoms to be more severe in middle-stage frontotemporal dementia. For example, you may notice an increase in reckless behaviors and impulsiveness. Or, if you experienced PPA in the early stages, you may now notice a worsening of language issues. 

It’s also possible you’ll begin to experience behavioral changes and symptoms of other variants. This means if you experienced behavioral symptoms in early-stage FTD, you may now begin to experience behavioral and language changes and issues in middle-stage FTD.

People with middle-stage frontotemporal dementia are likely to begin having difficulties with activities of daily living (ADLs) like getting dressed and bathing. 

Late-stage frontotemporal dementia

In late-stage FTD, symptoms continue to worsen, and the ADLs that started to be a struggle in middle-stage FTD are now increasingly difficult. A physician may prescribe antidepressants and antipsychotic medications to help make symptoms more manageable. 

Another sign of the end stage of frontotemporal dementia is memory loss. This is likely to include things like confusion, forgetting things that just occurred, and not recognizing loved ones. If you haven’t already, it is a good time to consider memory care options to help ensure the best-specialized care possible. 

Help for frontotemporal dementia caregivers

Caring for a loved one with FTD can be emotionally and physically challenging as you strive to ensure them the best care possible. We can help. Seniorly has done the research to give you the most reliable caregiving resources available.

Being aware of the resources available that meet your needs and budget will prove invaluable to you throughout your caregiving journey. 

Understanding the stages of frontotemporal dementia is vital for both patients and their loved ones. It's no surprise that this challenging neurological condition can have a profound impact on individuals.

Throughout this journey, remember that individuals with frontotemporal dementia are not defined by their condition. By embracing their evolving needs, familiarizing ourselves with the stages of this disease, seeking professional guidance, and offering unwavering support, we can help them navigate the challenges they face everyday life with grace and dignity.