Dementia: Not a Normal Part of Aging, Pt. 2

FTD is rare, and it tends to manifest at a younger age than other forms of dementia. Roughly 60% of people diagnosed with FTD are between 45 and 64 years old (National Institute on Aging, n.d.).

FTD is a progressive disease, meaning symptoms worsen over time. In the early stages of FTD, people may experience only one symptom. As the disease progresses, however, more parts of the brain are impacted, and other symptoms appear. 

FTD is not one single disease. In fact, it is a group of disorders. There are three types of FTD: behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and movement disorders. While each type of FTD is different, symptoms and the order in which they appear can vary from person to person. Additionally, the same symptoms can appear across each of the different types of FTD, and they can vary from one stage of illness to the next as different areas of the brain are affected. This can make it difficult to determine which FTD a person has.

Here is an introduction to the three types of FTD:

Behavioral Variant Frontotemporal Dementia (bvFTD)

This is the most common type of FTD. It involves changes in a person’s personality, behavior, and judgment. People with this disorder may have problems with cognition; however, their memory may stay relatively intact. Symptoms can include:

• Problems planning and sequencing

• Difficulty prioritizing tasks or activities

• Repeating the same activity or saying the same word over and over

• Acting impulsively and/or inappropriately without considering how others perceive the behavior

• Becoming disinterested in activities they used to care about

Over time, issues with language and movement may occur, causing the person with bvFTD to need increased care and supervision.

Primary Progressive Aphasia (PPA)

PPA involves changes in an individual’s ability to communicate. This includes his or her ability to use language to read, write, speak, and understand what others are saying. This includes difficulty using or understanding words (aphasia) and difficulty speaking properly (e.g., slurred speech). People with PPA may have one or both symptoms. Over time, they may lose their ability to speak at all, becoming mute.

Many people with PPA develop symptoms of dementia including problems with memory, reasoning, and judgment. As the disease progresses, some people with PPA may experience significant changes in behavior, similar to those seen in bvFTD. PPA normally comes on in midlife, before age 65

There are three types of PPA, categorized by the type of language problems that initially appear.

• Semantic PPA: A person slowly loses the ability to understand individual words and sometimes to recognize the faces of familiar people and common objects.

• Agrammatic PPA: A person develops increased trouble speaking and may omit words that link nouns and verbs in a sentence (such as to and from). Eventually, the person may no longer be able to speak at all. He or she may also develop movement symptoms similar to those seen in corticobasal syndrome.

• Logopenic PPA: A person has difficulty finding the right words during a conversation but retains an understanding of words and sentences. 

Movement Disorders

Corticobasal syndrome and progressive supranuclear palsy are two rare neurological movement disorders that are associated with FTD. They occur when the parts of the brain that control movement are affected. Thinking and language abilities may be affected as well. Other movement-related types of FTD include frontotemporal dementia with parkinsonism and frontotemporal dementia with amyotrophic lateral sclerosis (FTD-ALS).

Here is a brief synopsis of each of the four movement disorders associated with FTD:

• Corticobasal Syndrome: This movement disorder is caused by corticobasal degeneration — a gradual shrinkage and loss of nerve cells in certain parts of the brain. This degeneration causes a progressive loss in one’s ability to control movement. It often begins around age 60. One of the most prominent symptoms is apraxia, or the inability to use the hands or arms to perform movements, such as manipulating fasteners, despite having normal strength. Other symptoms can include muscle rigidity and difficulty swallowing. Symptoms may initially appear on one side of the body, but they will eventually affect both sides.

• Progressive Supranuclear Palsy: This movement disorder causes problems with walking and balance. People will often ambulate slowly, fall unexpectedly, experience a loss of facial expression, and have stiffness in the neck and upper body. A hallmark sign of this disorder is trouble with eye movements, giving the face a fixed stare. People may also develop problems with behavior, memory, and judgment.

• Frontotemporal Dementia with Parkinsonism: This movement disorder may be an inherited disease. Symptoms include movement problems like those of Parkinson’s disease, such as stiffness, slowed movement, and balance problems. People may also present with changes in behavior or language.

• FTD with Motor Neuron Disease (FTD-ALS): This movement disorder is a combination of bvFTD and amyotrophic lateral sclerosis (ALS), known colloquially as Lou Gehrig’s disease. People experience the behavioral and language changes seen in bvFTD along with the progressive muscle weakness seen in ALS. Symptoms of either disease may appear first, with other symptoms developing over time.

The cause of FTD is unknown in most cases. Individuals with a family history of FTD are more likely to develop FTD. About 10 to 30% of bvFTD is due to specific genetic causes (National Institute on Aging, n.d.) It is difficult to predict how long someone with FTD will live. Some people live less than two years post-diagnosis, while others live more than 10 years. Treatment for FTD focuses on symptom management, as there is currently no cure for FTD. Unfortunately, available treatments do not slow or stop the progression of the disease.

You might be wondering… how does FTD differ from Alzheimer’s dementia? According to the Alzheimer’s Association (n.d.), there are several key differences between FTD and Alzheimer’s:

1. Age at Diagnosis: Most people with FTD are diagnosed in their 40s and early 60s, while Alzheimer’s grows more common with increasing age.

2. Memory Loss: Memory loss tends to be a more prominent symptom in early Alzheimer's than in early FTD, although advanced FTD often causes memory loss as well as its more characteristic effects on language and behavior.

3. Behavioral Changes: Behavioral changes are among the first noticeable symptoms in bvFTD, the most common form of FTD. Behavioral changes occur with Alzheimer's as well, but they tend to occur later in the disease.

4. Problems with Spatial Orientation: Getting lost in familiar places is one example of a problem with special orientation. These symptoms are more common in Alzheimer's than in FTD.

5. Speech Problems: People with FTD often have trouble making sense when they speak, understanding the speech of others, and reading. Although people with Alzheimer's may have trouble thinking of the right word or remembering names, they tend to have less difficulty making sense when they speak compared to people diagnosed with FTD.

6. Hallucinations and Delusions: These symptoms are commonly experienced as Alzheimer's progresses, but relatively uncommon in FTD.

References

What are frontotemporal disorders? Causes, symptoms, and treatment. (n.d.) National Institute on Aging. https://www.nia.nih.gov/health/what-are-frontotemporal-disorders#:~:text=Frontotemporal%20disorders%20(FTD)%2C%20sometimes,work%2C%20or%20difficulty%20with%20walking.

Frontotemporal dementia. (n.d.) Alzheimer’s Association. https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/frontotemporal-dementia