Frontotemporal Dementia: Symptoms, Causes, and Treatment

CareLinx Team

July 6, 2017

13

min read

Article Highlights

This article will discuss the following topics:

What is Frontotemporal Dementia (FTD)?
Symptoms of Frontotemporal Dementia
Types of Frontotemporal Dementia
Causes of Frontotemporal Dementia
3 Main Types of FTD
Key Differences Between FTD and Alzheimer’s Disease (AD)
Diagnosis of Frontotemporal Dementia
Treatment and Management of Frontotemporal Dementia
Final Thoughts

What is Frontotemporal Dementia (FTD)?

Frontotemporal dementia is a group of related conditions resulting from the progressive degeneration of the frontal and temporal lobes of the brain. This disease is most commonly referred to as frontotemporal dementia (FTD), fronto-temporal lobar degeneration (FTLD), or Pick’s Disease. Damage in the frontal and temporal areas of the brain causes impairment and changes in personality, behavior, language, and muscle or motor function. Symptoms vary across individuals based on the parts of the brain that are affected and the stage of their disease progression. This variability can cause FTD to be misdiagnosed as a psychiatric problem or another form of dementia like Alzheimer’s disease.

FTD usually occurs in middle age (mid-40s to early-60s), and causes a steady, gradual decline in the ability to complete Activities of Daily Living (ADLs). ADL’s are often seen as markers of older adults’ ability to continue to live at home and maintain independence, and they include tasks like eating, bathing, dressing, and toileting. Thus, a decline in ability to perform ADL’s reflects a decline in functioning and a growing need for care.

Quick Fact:

FTD accounts for 20% of young-onset dementia cases.

Symptoms of Frontotemporal Dementia

The Frontal Lobes of the brain are associated with functions such as planning, reasoning, judgment, and problem-solving. Changes in the frontal lobe are generally associated with behavioral symptoms. The Temporal Lobes of the brain are involved with the processing of hearing, speech, emotion, and memory. Changes in the temporal lobes are linked to language and emotional disorders. As a result, different symptoms are present in each person with FTD as various parts of the frontal and temporal lobes are affected.

Behavioral Symptoms

Behavioral and personality symptoms consist of problems with executive function such as planning, multitasking, prioritizing, sequencing, and self-monitoring and correcting behaviors. Changes in behavior and personality may lead to a decreased concern for social norms, other people, and an overall lack of insight into one’s own behaviors. There are key words that describe some specific FTD behavioral symptoms:

Perseveration: the tendency to repeat the same activity or word
Social Disinhibition: acting impulsively without concern over how others may perceive the behavior
Compulsive Behavior: inability to inhibit urges to perform compulsive movements, such as repetitive checking behaviors
Utilization Behavior: difficulty in resisting impulses to use or touch objects

Emotional symptoms are noticeable through social exchanges such as difficulty reading and understanding social signs, facial expressions, and body language.

Individuals may also lack empathy, which may make them seem as though they are indifferent, selfish or uncaring. For example, if a close friend is in a terrible accident, an individual with FTD may not display any emotional reactions. Emotions may seem completely disconnected from a situation or expressed at the wrong time, or in the wrong context. They may also display apathy, and may not have the drive, initiative, or interest in doing certain things, including activities that previously brought them pleasure. Apathy is often incorrectly confused with depression.

Language Symptoms

As mentioned earlier, the temporal lobes are associated with language and emotion. Two distinct language symptoms are prevalent in those with FTD: aphasia and dysarthria.

Aphasia is a language disorder that is characterized by the inability to use and understand words. People with aphasia, however, may still be able to produce language that what may be comprehensible, but may lack clarity or proper understanding.
Dysarthria is a language disorder that impairs the physical ability to speak properly, causing pronunciation issues such as slurring words. Even though the ability to speak may be impaired, the message or point that an individual may be trying to convey remains intact.

Language problems get worse over time and progress at a quicker rate than other skills such as thinking or social behavior.

Motor Symptoms

Changes in the frontal lobe can initiate two main motor symptoms in FTD patients.

Dystonia is the abnormal posture of body parts such as the hands and the feet. For example, an individual’s hand can be bent or stiff and not be used in an activity that would normally require the use of two hands.
Apraxia is a gait disorder that causes abnormalities in walking, frequent falls, tremors, and clumsiness. Individuals with Apraxia are typically unable to perform common movements such as buttoning up a shirt or unzipping something, regardless of the physical strength that an individual may have. People may also experience neuromuscular weakness characterized by severe weakness and cramps in muscles.

Effects of FTD Symptoms

Difficulty in reasoning, judgment, organization, and planning can cause people with FTD to be gullible and fall prey to scams
Lack of judgement may lead to criminal behavior- shoplifting, indecent exposure, running stop lights, poor financial judgement, or impulsive buying
At the extreme, impulsivity can be self-destructive, such as trying to get out of moving cars

Quick Fact:

Only rough estimates are available, but there may be 50,000 to 60,000 people with bvFTD and PPA in the United States; the majority of whom are between 45 and 65 years of age.

Causes of Frontotemporal Disorders

In frontotemporal dementia, the frontal and temporal lobes of the brain shrink or atrophy. Typically, the cause is unknown. Certain genetic mutations have been linked to the development of specific subtypes of FTD, but more than 50% of individuals who develop FTD have no family history of the disease. That being said, other than family history of the disease, there are not currently any known risk factors for developing FTD.

3 Main Types of FTD

Frontotemporal dementia is characterized by three distinct types:

Behavioral Variant Frontotemporal Dementia (bvFTD)

Behavioral Variant Frontotemporal Dementia (bvFTD) is the most common type of FTD (about half of the cases). This condition causes changes in personality, behavior, and interpersonal relationships. Although bvFTD frequently occurs in individuals in their 50’s and 60’s, it can also develop in individuals in as early as in their 20’s, and as late as in their 80’s.

In bvFTD, the majority of nerve cell loss occurs in areas responsible for planning, behavior, self-control, empathy, and judgment, along with other abilities.. As a result, personality changes are inevitable. However, compared to Alzheimer’s disease, memory is usually relatively spared. Typical early symptoms consist of apathy or indifference, inappropriate and impulsive behaviors, excessive emotions, or loss of empathy or concern for others. This can be very difficult for family members to experience, but it is important to recognize that these changes are symptoms of the disease and your loved one is not acting this way on purpose.

Primary Progressive Aphasia (PPA)

Primary Progressive Aphasia (PPA), the second most common frontotemporal disorder, primarily influences language function, including speech, understanding others, naming common objects, and reading and writing. PPA usually develops in mid-life before the age of 65, but can also occur in later life. There are three sub-types of PPA that differ in terms of their major symptoms:
Semantic Subtype in which individuals develop problems with word-understanding or comprehension, Logopenic Subtype characterized by problems with word-finding, and Nonfluent or Agrammatic Subtype which consists of problems with word-order and word-production. PPA is caused by degeneration or decline in parts of the brain that affect speech and language. It typically begins very gradually and the first signs are usually difficulty thinking of common words while speaking or writing. However, like other forms of dementia, the disease is progressive, so it worsens over time to the point where individuals with PPA eventually experience extreme difficulty with any verbal communication.

Movement Disorders

There are also rare disorders associated with FTD that produce changes in motor and muscle functions with or without behavior or language difficulties.

Corticobasal Syndrome is a disorder that can cause the arms and legs to become uncoordinated or stiff, thus inhibiting motion. Symptoms may first appear on one side of the body, but eventually spread to both. This progressive loss of the ability to control movement occurs despite normal strength.
Progressive Supranuclear Palsy (PSP) is a disorder that can cause muscle stiffness, difficulty with walking and balance, and marked changes in posture. An early sign is often trouble with eye movements, especially looking down.
FTD-ALS (FTD with motor neuron disease) is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease. Symptoms typically include the behavioral and/or language difficulties that occur in bvFTD alongside the progressive muscle weakness characteristic of ALS. Either disease can develop first, with the additional symptoms occurring with time.
FTD with Parkinsonism is characterized by movement problems similar to Parkinson’s disease, such as stiff and slowed movement, difficulty with balance, and changes in behavior and language.

Individuals will likely experience progressive muscle weakness and coordination problems for many years. As a result, they can become wheelchair-bound or bed-bound. In addition, these physical challenges can also cause problems with chewing, swallowing, controlling bladder and bowels, and problems with moving about in general. These physical changes can ultimately lead to serious conditions such as urinary tract, skin, or lung infections, causing death.

Quick Fact:

Both bvFTD and PPA are far less common than Alzheimer’s Disease in those over the age of 65. However, in the 45 to 65 age range, bvFTD and PPA are nearly as common as younger-onset Alzheimer’s.

Diagnosis of Frontotemporal Dementia

There is no single test that can lead to a definitive FTD diagnosis. There are, however, two processes that can help lead to a diagnosis:

Confirmation through a genetic test in familial cases.
Conducting a brain autopsy once an individual is deceased

However, doctors can make a probable diagnosis. To do so, one of three doctors (a psychiatrist, psychologist, or neurologist) will usually collect information about the individual and perform appropriate tests. The doctor will compile individual and family medical histories, record symptoms, order testing if necessary to uncover genetic mutations, order blood tests, perform a physical exam, use brain imaging to look for changes in the temporal and frontal lobes, and conduct a neuropsychological evaluation to assess cognitive functions. Despite these measures, FTD may still be difficult to diagnose because its symptoms can be very similar to other conditions such as Alzheimer’s. Research is ongoing to discover a more accurate way to detect frontotemporal degeneration at an earlier stage.

Key Differences Between FTD and Alzheimer’s Disease (AD)

With that in mind, here are some useful tips for distinguishing between FTD and AD:

Age at dementia diagnosis (diagnosed in 40’s-early 60’s as opposed to AD which grows more likely with age)
Memory loss is more prevalent in early Alzheimer’s than in early FTD (though it may occur in advanced stages of FTD)
Behavioral changes are usually the first symptoms in bvFTD (these tend to occur later in people with AD)
Spatial orientation problems (such as getting lost) are more common in those with AD
Problems with speech, understanding, and reading are more common in FTD
Hallucinations and delusions are more common as AD progresses but not very common in FTD

Treatment and Management of Frontotemporal Dementia

Like Alzheimer’s and most other types of dementia, there are unfortunately no specific treatments that can cure any of the types of frontotemporal dementia. Unlike other types of dementia, there are also no treatments to slow down the progression of FTD. To make matters worse, due to the difficulty of the diagnosis, individuals may go a long time before they get any help in managing their symptoms. With the correct medications, however, behavioral symptoms like irritability, depression, and agitation can be better managed.

Even though medications to manage symptoms can help improve the person’s quality of life, FTD inevitably gets worse over time. As each person with FTD is different, the speed of decline and the manifestation of symptoms will vary. Symptoms of FTD progress at a rapid, steady rate. Individuals with frontotemporal dementia can survive between two to fifteen years and will most likely need 24-hour assistance with their Activities of Daily Living in their later years.

Quick Fact:

Changes in the patient’s behavior are often the first noticeable symptoms in bvFTD, the most common type of FTD.

Although Alzheimer’s Disease and FTD have some symptoms in common, the symptoms cannot be treated with the same pharmacological agents. This is because the cholinergic systems, which relate to nerve cells, are not affected in FTD as they are in Alzheimer’s. Moreover, the disinhibition and compulsive behaviors in an individual with FTD can be controlled by selective Serotonin Reuptake Inhibitors (SSRIs), commonly known as anti-depressants.

When caring for an individual with FTD, one must remember to always try to be sensitive, patient, and understanding. It is possible that they may say or do something that feels hurtful or unforgivable, but try to direct your anger at the disease instead of at the person. There are several things important to remember when providing care for someone with FTD. It is crucial to remember to care for yourself and make sure you get the breaks you need – seek assistance from other family members, friends, or hired help if possible. You may also wish to seek out a support group online or in your community to meet others going through similar experiences. You might also consider consulting with or hiring a professional who can help. For example, speech pathologists might be able to help your loved one with communication strategies, and occupational therapists may provide useful tips for redirecting behaviors and modifying daily activities to maintain engagement and independence for as long as possible. It can be helpful to limit choices and offer simple instructions and solutions for how to go about things. Caregivers may need to speak slowly and use simple sentences. Trying to maintain a regular schedule can also be helpful to reduce confusion and can have added benefits like improving sleep.

Final Thoughts

FTD can be a difficult disease for both the person with FTD and their family members and caregivers. The behavioral and personality changes that occur with the disease can sometimes cause family members to experience embarrassment and social alienation. However, it is important to recognize that individuals with FTD may be aware of and embarrassed by these changes as well. Anger and frustration, while potentially symptoms of the disease, may also be natural responses to the difficulties they are experiencing, being unable to perform basic activities or movements they previously used to do without thinking (e.g. tying or loosening shoelaces, following a recipe). Thus, it’s always important to imagine yourself in their shoes and provide the kind of care and unconditional love you would want to receive from your loved ones.