What is FTD?
Frontotemporal dementia (FTD) is a group of disorders characterized by progressive damage to the brain’s frontal and temporal lobes. It can affect people who are quite young — even in their 20s. The loss of nerve cells in these lobes cause deterioration in behavior, personality, and/or difficulty with producing or comprehending language. It is the most common form of dementia in individuals under sixty, affecting 50,000 to 60,000 people in the United States.
Like most forms of dementia, FTD is progressive. This means that its symptoms may be mild at first, but they will worsen over time. 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. In the advanced stages of FTD, individuals experience debilitating symptoms affecting various aspects of their cognitive and functional abilities, including loss of speech, muscle weakness, and difficulty swallowing. Advanced FTD also predisposes individuals to physical complications such as pneumonia, infections, or injuries resulting from falls.
Life expectancy depends on several factors, including the subtype of FTD, the age of onset, the specific symptoms, and the person’s overall health.
What is FTD?
Frontotemporal dementia (FTD) is a group of disorders characterized by progressive damage to the brain’s frontal and temporal lobes. It can affect people who are quite young — even in their 20s. The loss of nerve cells in these lobes cause deterioration in behavior, personality, and/or difficulty with producing or comprehending language. It is the most common form of dementia in individuals under sixty, affecting 50,000 to 60,000 people in the United States.
Like most forms of dementia, FTD is progressive. This means that its symptoms may be mild at first, but they will worsen over time. 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. In the advanced stages of FTD, individuals experience debilitating symptoms affecting various aspects of their cognitive and functional abilities, including loss of speech, muscle weakness, and difficulty swallowing. Advanced FTD also predisposes individuals to physical complications such as pneumonia, infections, or injuries resulting from falls.
Life expectancy depends on several factors, including the subtype of FTD, the age of onset, the specific symptoms, and the person’s overall health.
What causes FTD?
Scientists have examined the brains of patients who had various behavioral symptoms of FTD and found four stages of tau protein deposition in four regions of the brain:
- Stage 1: Frontotemporal limbic/paralimbic and neocortical regions, which control learning, memory, movement and judgement
- Stage 2: Subcortical structures including the basal ganglia, which is responsible for movement; locus coeruleaus, which is mainly responsible for arousal, vigilance and attention; and raphe nuclei, which produces serotonin
- Stage 3: Primary motor cortex and pre-cerebellar nuclei, both responsible for motor function
- Stage 4: Visual cortex, responsible for being able to see
In addition to tau, the protein TDP43 is present in the brains of some people with FTD. These two main proteins have also been found to be associated with Alzheimer’s disease. Read more about the research on TDP43’s role in dementia here.
Signs and symptoms of FTD
The symptoms of frontotemporal dementia fall into different clinical patterns that involve either changes in behavior, or problems with language. The two main subtypes are:
Behavioral variant frontotemporal dementia (bvFTD), commonly presents with changes in personality and behavior, including impulsivity, poor judgment, social disinhibition, and loss of interest in normal daily activities.
Signs and symptoms of FTD
The symptoms of frontotemporal dementia fall into different clinical patterns that involve either changes in behavior, or problems with language. The two main subtypes are:
Behavioral variant frontotemporal dementia (bvFTD), commonly presents with changes in personality and behavior, including impulsivity, poor judgment, social disinhibition, and loss of interest in normal daily activities.
Primary progressive aphasia (PPA) features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms. Some people also have physical symptoms with or without behavior (bvFTD) or language (PPA) problems, such as tremors, muscle spasms or weakness, rigidity, poor coordination and/or balance, or difficulty swallowing.
This is the kind of dementia that actor Bruce Willis has. Read is family’s account of his early signs.
Is FTD genetic?
FTD typically occurs sporadically, meaning that there is no known family history of FTD. In approximately 15 to 40% of FTD cases, a genetic mutation can be identified as the likely cause of the disease, and when this is inherited it is known as ‘familial FTD’.
The gene mutation rate can vary due to factors such as ancestry, family history, and the type of FTD. The majority of gene mutations are found in cases with a family history of neurodegenerative disease. Read more about FTD and genetics here.
Is FTD genetic?
FTD typically occurs sporadically, meaning that there is no known family history of FTD. In approximately 15 to 40% of FTD cases, a genetic mutation can be identified as the likely cause of the disease, and when this is inherited it is known as ‘familial FTD’.
The gene mutation rate can vary due to factors such as ancestry, family history, and the type of FTD. The majority of gene mutations are found in cases with a family history of neurodegenerative disease. Read more about FTD and genetics here.
What’s the difference between FTD and Alzheimer’s disease?
One thing that looks different: the symptom of aggression. For people with frontotemporal dementia, physical aggression started early in their disease. The frontal parts of the brain control things like empathy, impulse, personality and judgment. Alzheimer’s, on the other hand, is located further back in the brain, in an area associated with memory and orientation in space and time. Research has found that people living with Alzheimer’s are more likely to be physically aggressive, though that behavior was less frequent, less violent, and less likely to be directed at strangers than behavior observed in those with FTD. Read more about the research.
One thing that looks different: the symptom of aggression. For people with frontotemporal dementia, physical aggression started early in their disease. The frontal parts of the brain control things like empathy, impulse, personality and judgment. Alzheimer’s, on the other hand, is located further back in the brain, in an area associated with memory and orientation in space and time. Research has found that people living with Alzheimer’s are more likely to be physically aggressive, though that behavior was less frequent, less violent, and less likely to be directed at strangers than behavior observed in those with FTD. Read more about the research.
Learning resource
- What are FTD’s symptoms and characteristics?
- What causes FTD?
- What’s the difference between FTD and Alzheimer’s?
- What’s the latest research on FTD?
- and more
Experts talk FTD in Being Patient’s Brain Talks
Katie Brandt and Dr. Brad Dickerson: Understanding frontotemporal dementia
FTD is far less common than Alzheimer’s disease, but is similar in development as a neurodegenerative disease. The disease gradually progresses to cause symptoms in the patient likely some number of years after the disease began in the brain. FTD tends to have a younger age of onset, focused on degeneration of the frontal and temporal lobes of the brain, residing in the front of the skull. Alzheimer’s typically focuses on degeneration in the back parts of the brain. Read more here.
Slowing down the symptoms of FTD
UCSF neuropsychologist Kaitlin Casaletto on how to slow FTD progression
Dr. Kaitlin Casaletto, neuropsychologist and assistant professor at UCSF’s Memory and Aging Center, discusses research on slowing the progression of frontotemporal dementia. In a Live Talk, she digs into the science behind how a more active lifestyle is associated with slower disease progression. Check out this article or read and watch her Live Talk.
Patient Voices
Laurie Scherrer: Living well with a combination of early-onset Alzheimer’s and FTD
Writer, advocate, and international speaker Laurie Scherrer speaks on her experience as a person living with an FTD and early-onset Alzheimer’s diagnosis. Scherrer offers the wisdom, “you need to constantly focus on your abilities, not your inabilities.” Her diagnosis began with mild difficulty with mathematics and forgetting entire conversations. Laurie Scherrer balanced career, family, her personal life and diagnosis and has turned towards community education and advocacy. Read more here.
Emily Ong: Misdiagnoses and dementia advocacy in Asia
Emily Ong, who lives with FTD, shares her vision of forging a more open and compassionate society for those living with dementia. Ong says of her advocacy efforts, “I’m very happy that there are more and more people, even women who are living with dementia — young people like me — who are also stepping up and sharing in Chinese. I feel that that is very worthwhile for all the sacrifices that I make.”
Scott Rose: Love, Companionship and Dementia
Finding moments to be grateful for — those precious but fleeting moments — can be so difficult with the changes that accompany dementia. Scott Rose challenged himself to find these moments while caring for his wife Maureen Patrick-Rose who lived with FTD and appendiceal cancer before her passing in 2019.
FTD at 29: Dawn Kirby and Dr. Brad Dickerson on Caregiving in Frontotemporal Dementia
Dawn Kirby, shares her experience caring for her daughter, Kara Kirby, who was diagnosed with FTD at the age of 29. Dr. Brad Dickerson, director of the Frontotemporal Disorders Unit at Massachusetts General Hospital, joined Being Patient to speak about FTD diagnosis, treatment, and care of people living with the condition.
Treating FTD
Testing a drug’s disease-modifying potential for FTD
A phase 2a clinical trial is underway to test the possible benefits of sodium selenate for people living with frontotemporal dementia. Read more here.
Clearing tau in FTD: researchers identify target protein
Researchers have pinpointed a protein that contributes to tau accumulation in the brain, marking it as a possible therapy for FTD. Read more here.
Researchers explore how antibiotics may help treat FTD
In a new study, researchers found that an antibiotic class called aminoglycosides could be a promising treatment for frontotemporal dementia. Read more here.
Is FTD preventable?
Research into protection against FTD
Neurologist Luke Harper at Lund University describes new advances in research into frontotemporal dementia. The brain gains its distinctive folds in the womb, which play a critical role in our later cognitive function. Research found some people have a protective feature in the brain that may delay the onset of FTD dementia symptoms. Read more here.
Evidence Staying Active Slows Onset of FTD
Scientists are continuing to search for answers to Alzheimer’s disease and other dementias, targeting beta-amyloid and tau protein, inflammation, and genetic factors in research on potential therapies. But more and more, doctors are turning to something quite simply and straightforward as part of the answer to dementia—staying active. Read more here.
