Frontotemporal Dementia
Frontotemporal dementia is a group of brain diseases that mainly affects the frontal and temporal lobes, causing changes in personality, behavior, language, and sometimes movement. It differs from other forms of dementia because it typically strikes people at a younger age, often between 45 and 65, affecting individuals during their working years and while raising families.
Table of contents
- What is Frontotemporal Dementia?
- Types of Frontotemporal Dementia
- Symptoms
- Causes and Risk Factors
- Diagnosis
- How the Disease Progresses
- Treatment and Management
- Caring for Someone with FTD
What is Frontotemporal Dementia?
frontotemporal lobar degeneration (FTLD), Pick’s disease
Frontotemporal dementia (FTD) is an umbrella term for a group of brain diseases that mainly affect the frontal and temporal lobes of the brain. These are the areas behind your forehead and behind your ears. These parts of the brain control personality, behavior, and language skills[1].
In frontotemporal dementia, parts of these lobes shrink, a process known as atrophy. This happens when nerve cells (called neurons) in these parts of the brain are damaged and die. As more cells lose function, the frontal and temporal lobes get smaller[2].
- Frontal lobes
- Temporal lobes
FTD is different from Alzheimer’s disease in several important ways. It typically occurs at a younger age than Alzheimer’s disease, most often beginning between the ages of 40 and 65, although it can occur later in life as well. About 60% of people with FTD are between 45 and 64 years old when they develop the condition[4]. FTD is the most common form of dementia for people under age 60[3].
Because FTD strikes younger people, it has a substantial impact on work, family, and finances. The economic burden of FTD is approximately $120,000 per year, nearly double the amount associated with Alzheimer’s disease[3]. FTD accounts for about 10% to 20% of all dementia cases[1].
Types of Frontotemporal Dementia
There are several types of FTD, depending on which part of the brain is affected and what symptoms appear[2]:
Behavioral variant FTD (bvFTD) is the most common type. Damage to the frontal lobes affects behavior and personality. People may act inappropriately in social situations, lose empathy, or develop unusual eating habits. They may not be aware their behavior is inappropriate[2].
Primary progressive aphasia (PPA) involves changes in the ability to communicate, speak, read, write, and understand what others are saying. Damage to the temporal lobes affects language and communication skills. There are different variants of PPA, including nonfluent/agrammatic variant, semantic variant, and logopenic variant[3].
Movement-related types of FTD cause problems with physical movement. Progressive supranuclear palsy affects eye movements, balance, and walking. Corticobasal syndrome (also called corticobasal degeneration) causes problems with movement, like difficulty using one side of the body along with changes in thinking[2].
Frontotemporal dementia with parkinsonism affects movement, language, behavior, balance, and muscle control[2].
FTD-ALS is a combination of FTD and amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s disease). It affects behavior and language skills along with progressive muscle weakness and muscle jerks[2].
Symptoms
Symptoms of frontotemporal dementia differ from one person to the next and depend on which part of the brain is affected. Symptoms get worse over time, usually over years. People with FTD tend to have clusters of symptom types that occur together[1].
The most common symptoms involve extreme changes in behavior and personality. These include increasingly inappropriate social behavior, such as acting without a filter when speaking, lack of respect for others, or participating in risky behaviors. Loss of empathy and other interpersonal skills is common—for example, not being sensitive to another person’s feelings or having difficulty reading the emotions of others[1].
Many people develop apathy, meaning no motivation, social isolation, or a lack of self-care and hygiene. Changes in food interests are common, including overeating or eating things that aren’t food (called pica). Repeated behaviors, such as saying or doing the same things over and over, can occur[2].
Loss of executive function makes it difficult to plan, solve problems, and stay organized. Unlike Alzheimer’s disease, memory problems tend to occur later in FTD rather than early on[8].
Language problems can include not having the right words to say, mixing up words, losing the ability to read and write, or speaking slowly and struggling to make the right sounds when saying a word[2].
Movement changes may include muscle stiffness, weak muscles, slowed movements, or jerking muscle movements. Problems with balance, walking, eye movements, and difficulty swallowing can develop[2].
Symptoms may start mild and will get worse over time. Often, people with FTD appear physically healthy despite the changes happening in their brain[3].
Causes and Risk Factors
FTD happens when nerve cells in the frontal and temporal parts of the brain die. Proteins group together within the cells and damage them. The two most common proteins involved are called tau and TDP-43. For reasons that are not yet fully understood, these proteins have a preference for the frontal and temporal lobes[5].
In some cases, changes or differences in genes can be identified as the cause of FTD. About 10% to 30% of behavioral variant FTD cases can be attributed to genetic causes. Studies show that there may be a genetic variant that causes some cases of the disease. This is a change to the instruction manual that DNA uses to do a specific job in the body[2][6].
You may be more at risk of developing FTD if it runs in your biological family history or if you had a traumatic brain injury. People with a family history of FTD are more likely to have the condition[2][6].
In many cases, the exact reason why FTD happens isn’t fully understood. Research is ongoing to learn more[2].
Diagnosis
There is no single test for frontotemporal dementia. Healthcare professionals consider your symptoms and exclude other possible causes. FTD can be hard to diagnose early because symptoms often overlap with those of other conditions[9].
FTD is often misdiagnosed as a mental health condition, Alzheimer’s disease, depression, or Parkinson’s disease. On average, it currently takes 3.6 years to get an accurate diagnosis[3].
Healthcare professionals may order blood tests to help rule out other conditions, such as liver or kidney disease. If you experience loud snoring and pauses in breathing while you sleep, you may need a sleep study. Some symptoms of obstructive sleep apnea can be similar to those of FTD[9].
An assessment of symptoms is normally helpful, and it’s usually important to have somebody who knows the person well give an account of their symptoms. This is especially true because someone with FTD may not be aware of changes in their behavior[8].
Healthcare professionals may test reasoning and memory skills through neuropsychological testing. This type of testing is especially helpful to learn which type of dementia you may have at an early stage[9].
Brain scans can reveal visible conditions and show changes in the shape or size of the frontal or temporal lobes. Images of the brain may be taken using magnetic resonance imaging (MRI), which uses radio waves and a strong magnetic field to produce detailed images. A CT scan (computed tomography scan) or PET scan (positron emission tomography scan) may also be used. A PET scan uses a low-level radioactive tracer that can help show areas of the brain where nutrients are poorly metabolized[9].
A lumbar puncture may be performed to test the spinal fluid. This may be useful to rule out Alzheimer’s disease as the cause of symptoms[8].
How the Disease Progresses
Frontotemporal disorders are progressive, meaning symptoms get worse over time. In the early stages, people may have just one type of symptom. As the disease progresses, other symptoms will appear as more parts of the brain are affected[4].
The progression of symptoms—in behavior, language, and movement—varies by individual, but FTD brings an inevitable decline in functioning. The length of progression varies from 2 to over 20 years. Some people live more than 10 years after diagnosis, while others live less than two years after they are diagnosed[3][4].
Average life expectancy is 7 to 13 years after the start of symptoms. The average survival time after symptoms start is around 8 to 10 years[3][8].
As the disease progresses, the person affected may experience increasing difficulty in planning or organizing activities. They may behave inappropriately in social or work settings and have trouble communicating with others or relating to loved ones[3].
As FTD gets worse, complications can include agitation or aggression, delusions or hallucinations, difficulty swallowing, loss of all communication or language skills, loss of muscle control, memory loss, mental health challenges like depression, and trouble recognizing loved ones[2].
Over time, FTD predisposes a person to physical complications such as pneumonia, infection, or injury from a fall. The most common cause of death is pneumonia[3].
Treatment and Management
Today, there is no cure for FTD, and no treatments available to slow or stop the progression of the disease. However, there are ways to help manage the symptoms[3][4].
Before treatment starts, current and future health and social care needs should be assessed, and a care plan drawn up. This is a way of ensuring you receive the right treatment for your needs. It involves identifying areas where you may need some assistance[10].
Medicines cannot stop frontotemporal dementia getting worse, but they can help reduce some of the symptoms for some people. Antidepressants called selective serotonin reuptake inhibitors (SSRIs) may help control the loss of inhibitions, overeating, compulsive behaviors, apathy, depression, agitation, anxiety, and obsessive-compulsive behaviors. These medicines are considered relatively safe in the dementia population[10][12].
Antipsychotic medicines are rarely used, but are sometimes needed if SSRIs have not worked. They can help control severely challenging behavior that’s putting the person with dementia or others around them at risk of harm. Use of these medicines is limited due to concerns regarding side effects[10][11].
Several types of FTD cause problems with movement. Medications and physical and occupational therapy may provide modest relief for movement symptoms. Compared to Parkinson’s disease, FTD-related movement problems are generally not responsive to dopamine replacement therapies, but a small percentage of patients may experience improvement with a trial of carbidopa-levodopa[11][15].
For FTD-ALS, a medication called riluzole is approved and indicated as therapy[11].
In addition to medicines, there are a number of therapies and practical measures that can help make everyday living easier for someone with dementia. Occupational therapy can help identify problem areas in everyday life, such as getting dressed, and help work out practical solutions. Speech and language therapy can help improve communication or swallowing problems and determine the best tools and strategies. Physiotherapy can help with movement difficulties[10][15].
Other approaches include relaxation techniques such as massage and music or dance therapy, social interaction and leisure activities, and strategies for challenging behavior such as distraction techniques, a structured daily routine, and activities like doing puzzles or listening to music[10].
It is important for people with FTD to stay engaged in enjoyable, meaningful, and stimulating activities of their choosing as long as they are able and it is safe to do so. As FTD progresses, adjustments need to be made based on the person’s abilities and goals. Seek ways to adapt interests and hobbies to the person’s current needs and provide additional support and supervision as needed[16].
Research suggests that a physically and mentally active lifestyle may help. Even people with a genetic predisposition for FTD can take actions to increase their chances of living a long and productive life. Exercise and cognitive fitness are among the best ways to help manage the condition[17].
Caring for Someone with FTD
Caring for someone with FTD can be hard, both physically and emotionally. Caregivers may face challenges with managing medical and day-to-day care, as well as changing family and social relationships, loss of work, poor health, stress, decisions about long-term care, and end-of-life concerns[15].
When managing behavior changes, try to recognize it’s the illness talking and accept rather than challenge people with behavioral symptoms. Arguing or reasoning with the person will not help; they cannot control their behaviors or even see that their behavior is unusual or upsetting to others[15].
To help with language issues, speak slowly and clearly, use simple sentences, wait for responses, and ask for clarification if you don’t understand something. Gesturing, drawing, and using an album with labeled photos of people and objects may help communicate without talking[15].
Try to keep a routine, such as bathing, dressing, and eating at the same time each day. Help the person write down to-do lists, appointments, and events in a notebook or calendar. Plan activities that the person enjoys and try to do them at the same time each day. When dressing or bathing, allow the person to do as much as possible[18].
Home-based help will usually be needed at some stage, and some people will eventually need care in a nursing home[8].
It may be helpful to get in touch with a support group. Support groups offer an emotionally supportive environment in which to discuss care challenges and approaches. Connecting with others who understand FTD and hearing about their experiences is extremely helpful[10][16].
If you’ve been diagnosed with dementia, you might want to make arrangements for your care that take into account the decline in mental abilities. This may include ensuring your wishes are upheld if you’re not able to make decisions for yourself. You may want to consider drawing up an advance decision, having a plan for where you want to receive treatment as your condition becomes more advanced, or giving a relative lasting power of attorney[10].
