Frontotemporal Dementia: What Caregivers Can Expect

A man with dementia.

As a primary caregiver of a loved one with frontotemporal dementia (FTD), it can be difficult navigating the points between the diagnosis and knowing what to expect on your journey. With chronic illnesses and various forms of dementia on the rise, many of us will likely be tasked with taking care of a loved one or family member at some point in our lives.

According to The Association for Frontotemporal Dementia, an estimated 250,000 Americans have FTD, which counts for 10-20 percent of all dementia cases. It is known as one of the most prevalent types of presenile dementia, affecting people of younger or middle age. The onset of FTD can happen as young as 40 years of age and affects both genders equally.

Getting as informed as possible is the first step in learning to manage FTD as a caregiver. The earlier you can do this, the more you’ll be prepared for the arduous journey ahead and have an idea of what to expect. Managing expectations counts for a large part of the effort, helping you to look after yourself and your loved one to the best of your ability.

In this article, we’ll cover what frontotemporal dementia is, symptoms and methods for diagnosis, and actionable advice you can implement as a close caregiver. Caregiving isn’t easy by all accounts, but knowing your options and planning can make all the difference.

What Is Frontotemporal Dementia?

Frontotemporal dementia, otherwise known as Frontotemporal Degeneration, Frontotemporal Lobar Degeneration (FTLD), Frontal Lobe Dementia, or Pick’s disease, is a group of disorders that causes progressive degeneration (or atrophy — wasting away) of nerve cells in the brain. Particularly targeted are the frontal and temporal lobes, areas that are responsible for behavior, movement, and personality. Not only do those affected experience a change of personality and behavior, but there is also a progressive loss of speech and language skills. It is also possible to experience tremors and spasms.

This type of dementia is very distressing to witness as a loved one because of the behavioral changes associated with the disease. The person you knew so well can suddenly become aggressive, talk inappropriately, lose their ability to coordinate, and a whole host of other functional disabilities.

FTD can manifest in various types. Subtypes of FTD are identified clinically according to the symptoms that appear first and most prominently. Some of the most common types of FTD are:

  • Frontal/behavioral variant frontotemporal dementia(bvFTD). This type of FTD presents the more typical manifestations associated with the disease, such as character changes and behavioral issues. This is the most common type. It can also affect language and thinking skills. 
  • Primary progressive aphasia (PPA). This type of FTD is commonly divided into two secondary forms:
  • Non-fluent variant (nfvPPA). This subtype affects speech ability. Those affected with this variant find it increasingly difficult to speak, though they still recall the understanding of individual words. They suffer from apraxia of speech. They may be physically struggling for their words.
  • Semantic variant (svPPA). This subtype affects knowledge and the use of language. It is characterized by the progressive loss of the meaning of words. If there is also a problem with being able to identify people or objects, this is called semantic dementia. Their speech becomes vague as they omit or substitute words they don’t understand.
Frontotemporal dementia is the 5th most common cause of dementia.

The Causes Of Frontotemporal Dementia

There is no known cause of frontotemporal dementia. In some people suffering from FTD, some substances accumulate in their brain cells — very small structures called Pick bodies. These contain an abnormal amount of protein called tau and TDP-43 proteins, which disrupt the brain cell’s ability to communicate with other cells. It is unknown why these proteins suddenly accumulate in such large numbers.

One of the most plausible theories is linked to genetics. About 10 to 15 percent of people with FTD have a family history of dementia. Research has shown that various subtypes of FTD are caused by gene mutations and can be inherited. The most common FTD variant that can be inherited is the behavioral variant, and less common in the semantic variant.  However, this is still unproven as the majority of people diagnosed with FTD have no family history of the disease or related dementias.

There does seem to be a link between frontotemporal dementia and amyotrophic lateral sclerosis (ALS) also known as Lou Gehrig’s disease or motor neurone disease. It has been found that the C9orf72 gene is the most common gene causing hereditary FTD, ALS, and ALS with FTD. ALS is an incurable neurodegenerative disease affecting the loss of upper and lower motor neurons. This leads to paralysis, dysphagia, dysarthria, and eventually to respiratory failure. A small number of people affected by FTD also develop ALS. However, more research needs to be done to confirm the shared genetics and molecular pathways.

What Is The Difference Between Frontotemporal Dementia And Other Types of Dementia?

Frontotemporal Dementia is separate from other common dementias and illnesses such as Alzheimer’s Disease, though can often be misconstrued because of overlapping symptoms. FTD sets itself apart from the rest in a few distinct ways. One of the main differences is that the onset of FTD starts at a younger age than the average sufferer of dementia. First symptoms usually begin to appear in middle age, around 50 years old. It takes time for the symptoms to develop, often up to 10 years before FTD is diagnosed. This is because the symptoms are usually thought to be a sign of mental illness, affecting the person’s work and home life in the process.

A surefire sign of FTD is a progressive deficit in social behavior and gradual loss of language — not impaired memory, but slurs and difficulties with expression. As expected with these types of symptoms, life for caregivers becomes very difficult and relationships tend to get under a lot of strain.

What To Expect With Frontotemporal Dementia

Frontotemporal dementia life expectancy ranges from 2 to over 20 years, with an average course of 8 years from the onset of symptoms. Because of the nature of these symptoms (and the fact that a patient is often “too young” for dementia to be considered), FTD is often initially misdiagnosed as a psychiatric problem or movement disorder, such as Parkinson’s disease. Alzheimer’s disease is another possible misdiagnosis. Accurate diagnosis is crucial, as some medications used to treat other disorders may be harmful in a person with FTD.

Existing care facilities and programs may not be appropriate for—indeed, many do not accept–younger individuals as patients without additional education and support about FTD. To accurately diagnose your loved one, pay attention to any symptoms that may occur.

Symptoms of frontotemporal dementia: Symptoms of FTD start gradually and progress steadily, and in some cases, rapidly. They vary from person to person, depending on the areas of the brain involved. These are common symptoms:

  • Socially inappropriate, impulsive, obsessive or repetitive behaviors
  • Unusual verbal, sexual, or physical behavior
  • Weight gain due to dramatic overeating
  • Impaired judgment
  • Apathy
  • Lack of empathy
  • Decreased self-awareness
  • Behavior and/or dramatic personality changes, such as swearing, stealing, increased interest in sex, or a deterioration in personal hygiene habits
  • Loss of interest in normal daily activities
  • Emotional withdrawal from others
  • Loss of energy and motivation
  • Inability to use or understand language; this may include difficulty naming objects, expressing words, or understanding the meanings of words
  • Hesitation when speaking
  • Less frequent speech
  • Distractibility
  • Difficulty planning and organizing
  • Frequent mood changes
  • Agitation
  • Increasing dependence

Some people have physical or psychiatric symptoms, such as tremors, muscle spasms or weakness, rigidity, poor coordination, and/or balance, difficulty swallowing, hallucinations, or delusions. These signs are not as common as behavioral and language changes.

Family members are often the first to notice subtle changes in a person’s behavior or language skills. A person needs to see a doctor as early as possible to discuss:

  • Symptoms, including which symptoms, when they began, and how often they occur
  • Medical history and previous medical problems
  • Medical histories of family members
  • Prescription medications, over-the-counter medicines, and dietary supplements taken

Diagnosis of Frontotemporal Dementia

No single test can diagnose FTD. Typically, doctors will order routine blood tests and perform physical exams to rule out other conditions that cause similar symptoms. If they suspect dementia, they may:

  • Evaluate the person’s neurological health–reflexes, muscle strength, muscle tone, sense of touch and sight, coordination, and balance.
  • Assess the person’s neuropsychological status–memory, problem-solving ability, attention span, and counting skills–and language abilities.
  • Order magnetic resonance imaging (MRI) or computed tomography scans of the brain.
  • Order lumbar puncture, also called a spinal tap, to rule out any rare infections, inflammatory processes, or cancers that mimic FTD.

Frontotemporal Dementia Stages

Once diagnosed, it may be helpful to determine a loose timeline as to the progression of the disease. As mentioned before, FTD has an average course of 8 years. However, every case is different and there is no set timeline for each person. To gauge some semblance of a timeline, it is helpful to determine what stage they may be in. Here is an outline of each FTD stage:Early-stage frontotemporal dementia:

Behavioral variant: may disregard normal social boundaries or do things seen as inappropriate. They are impulsive, careless, and even commit crimes. Empathy and judgment are lacking as the person begins to become apathetic.

Semantic variant: forgets names of people, places, and objects. Has trouble finding the right word or remembering what a word means. May have behavior issues as well such as trouble sleeping, irritability, emotional withdrawal, and depression.

Non-fluent variant: Instead of forgetting individual words, grammar becomes difficult and misused. Those affected have labored and halting speech. 

Middle stage frontotemporal dementia:

As FTD progresses, the symptoms start to resemble those of Alzheimer’s patients and other dementias. In this stage, those with the behavioral variant will likely need more help with activities of daily living, or ADLs. This could include dressing, toileting, bathing, etc. They also may start to have more language difficulties. Similarly, those variants who started with language difficulties will start to develop behavioral issues.

Late-stage frontotemporal dementia:

This stage especially resembles those of Alzheimer’s patients. Language and behavior are both issues, as well as memory deterioration and possibly memory loss. At this point, constant care may be necessary. This is to ensure the comfort and care of the patient. It is possible for the affected to die of an infection such as pneumonia at this stage.

Talk with your healthcare providers about when to call them. Your healthcare provider will likely advise calling if symptoms become worse, or if there are obvious or sudden changes in behavior, personality, or speech. This includes mood changes, such as increasing depression or feeling suicidal. Here are some tips to help you get the most from a visit to your healthcare provider:

  • Know the reason for your visit and what you want to happen.
  • Before your visit, write down the questions you want to be answered.
  • Bring someone with you to help you ask questions and remember what your provider tells you.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also, write down any new instructions your provider gives you.
  • Know why a new medicine or treatment is prescribed, and how it will help you. Also, know what the side effects are.
  • Ask if your condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if you do not take the medicine or have the test or procedure.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your provider if you have questions.
Fortunately, there are different treatments for Frontotemporal dementia.

Types Of Treatments Available

Currently, no treatments are available to cure or slow the progression of FTD. Doctors may prescribe medications to treat symptoms. Antidepressant medications may help treat anxiety and control obsessive-compulsive behaviors and other symptoms. Prescription sleeping medications can help ease insomnia and other sleep disturbances. Antipsychotic medications may reduce irrational and compulsive behaviors.  

Little solid evidence exists that antioxidants and other supplements help those with FTD. You and your family members should talk with your healthcare provider about whether to try antioxidants and other supplements, such as coenzyme Q10, vitamin E, vitamin C, and B vitamins to support brain health.

There are many forms of therapies that can assist a person to adjust to some of the changes caused by FTD. Some of those include:

  • Occupational therapy: Occupational therapists can help the patient identify problem areas in life, like getting dressed, and help work out solutions.
  • Speech and language therapy: Speech therapy can help improve language problems and swallowing problems.
  • Physiotherapy: This will help with movement problems.
  • Relaxation therapy: This includes massage, music, or dance therapy.
  • Social interaction/leisure: Memory cafes are held for dementia patients with memory issues to get support and advice. 

What You Can Do as a Caregiver

Coping with FTD can be frightening, frustrating, and embarrassing for both the person with the disease and family members. Since some symptoms can’t be controlled, family members shouldn’t take their loved one’s behaviors personally. Families need to maintain their well-being while ensuring that their loved one is treated with dignity and respect.

Caregivers should learn all they can about FTD and assemble a team of experts to help the family meet the medical, financial, and emotional challenges they are facing. It’s important to find a doctor knowledgeable about FTD. Other health care specialists who may play a role on the team are home-care nurses, neuropsychologists, genetic counselors, and speech and language, physical, and occupational therapists. Social workers can help patients and caregivers find community resources, such as medical supplies and equipment, nursing care, support groups, respite care, and financial assistance.

As a caregiver, it is important to begin to plan for what is to come. Attorney and financial advisors can help families prepare for the later stages of the disease. Advanced planning will help smooth future transitions for the person and family members and may allow the afflicted person to participate in the decision-making process.

It can be very stressful for a caregiver to take care of a loved one with FTD. It’s normal to have feelings of denial, anger, and irritability. Caregivers may also have anxiety, depression, exhaustion, and health problems of their own. Caregivers should contact their healthcare provider if they have any of these signs of stress. Keep in mind that caregiving for others takes away from time you care for yourself. Try to take time out for self-care, which will end up benefiting you and your loved one.

We hope that you now have a better understanding of frontotemporal dementia and that you have gained a little more confidence to get yourself through this arduous journey. By knowing what you can expect and the resources available to help your loved one, you can map out a plan to make it as smooth as possible. 

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