What is it?
Frontotemporal dementia (FTD), sometimes also known as frontotemporal lobar degeneration, is one of the most common causes of early-onset dementia. FTD refers collectively to three common clinical syndromes:
- behavioral variant frontotemporal dementia (bvFTD)
- non-fluent variant primary progressive aphasia (nfvPPA), and
- semantic dementia (also known as semantic variant primary progressive aphasia [svPPA])
The brain areas affected by FTD—the frontal and anterior temporal lobes—control reasoning, decision-making, motivation, social graces, behavioral control, personality, emotion, movement, speech, language, and some aspects of memory.
FTD is marked by dramatic changes in personality, behavior, and some thought processes. Changes in personality and social conduct occur in early stages of the disease, including disinhibited and impulsive behavior, apathy, social withdrawal, repetitive/compulsive behavior, and dietary changes. These symptoms may lead to misdiagnosis as a psychological or emotional problem; in the elderly, it can be mistaken for withdrawal or eccentricity.
Like other types of dementia, FTD usually develops slowly and gets gradually worse over several years. In later stages, people can have significant loss of expression, difficulty speaking, and immobility.
How common is frontotemporal dementia? Who gets it?
As many as 7 million Americans may be afflicted with a form of dementia. FTD may account for 2 percent to 5 percent, or 140,000–350,000, cases of dementia, and for as many as 25 percent of dementias that develop before age 65.
FTD affects men and women equally, and onset is usually after age 40 and before age 65. Approximately 40 percent of people diagnosed with FTD have a family history that includes at least one other relative with dementia. There is a strongly inherited genetic link in at least 10 percent of people diagnosed with FTD.
Additionally, a form of dementia in persons with motor neuron disease (amyotrophic lateral sclerosis, commonly known as ALS or “Lou Gehrig’s Disease”) may be associated with FTD.
Does my friend or family member have it?
Initial symptoms of FTD usually include changes in personality and behavior. Still, they vary from person to person, and they depend on the severity and location of the changes in the brain. In most cases, changes to the temporal lobes affect emotions and language, while changes to the frontal lobe often lead to behavioral symptoms and executive function difficulties.
Early on, symptoms can be missed or misunderstood by friends and family. The person you care for may do things and act in ways that are out of character. It’s important to remember that the person with FTD doesn’t have control over these shifts in behavior. They may be unable to recognize their illness and the limitations it brings (known as anosognosia).
Symptoms of FTD can include the following:
- Apathy. Lack of initiative and difficulty starting projects, tasks, or activities; lack of interest; or lack of concern. They may neglect domestic, financial, and occupational responsibilities.
- Emotional changes. The person’s emotional expression may be shallow (e.g., flat facial expression or lack of emotional response), exaggerated, or inappropriate (e.g., singing, dancing, clapping, or reciting phrases repeatedly).
- Social-interpersonal changes. Someone with FTD may struggle with sympathy or empathy. They can have difficulty reading facial expressions and social cues. They may have a diminished response to other people’s needs and feelings and decreased social interest, interrelatedness, or personal warmth.
- Disinhibition. Socially inappropriate behavior, such as laughing loudly in church, or exhibiting behavior others would not understand or appreciate, like singing or humming in the middle of a work meeting. Other types of behavioral disinhibition include loss of manners and impulsive, rash, or careless actions. The person usually doesn’t perceive these actions as personal or social misconduct.
- Executive functioning challenges. Struggling with planning, prioritizing, decision-making, and multi-tasking. They also likely have trouble monitoring themselves, adjusting, and correcting their own behavior. There can be rigid and inflexible thinking and impaired judgment.
- Perseveration. Repeating the same word or phrase or doing the same activity over and over.
- Compulsions. Repetitive, ritualistic behaviors can sometimes become complex routines. For example, the person with FTD may have rituals of grooming and dressing, while at the same time neglecting proper hygiene.
- Hyperorality and dietary changes. The person may binge on food, sometimes taking food from another person’s dish, and increase consumption of alcohol or cigarettes. They may have altered food preferences, such as an increased preference for sweets and junk food. Oral exploration or consumption of inedible objects is also possible.
- Utilization behavior. Grabbing objects within reach and starting the appropriate behavior/action associated with the object at an inappropriate time. For example, when conducting an office meeting, the person may see a tape dispenser nearby and begin pulling out strips of tape even if there is no actual need for the tape.
- Aphasia. The person continues to have the physical ability to speak, but they are unable to understand or use words correctly, fluently express themselves, or use grammar correctly. Someone with aphasia may not comprehend what you say to them. When they speak, they might repeat words or substitute words or syllables. Their speech may not make complete sense.
- Dysarthria. Weakness or difficulty controlling the muscles used to speak. Dysarthria causes slurred or slow speech that is difficult to understand.
- Rigidity. Inability of the muscles to relax normally.
- Dystonia. Involuntary muscle contractions that cause slow repetitive movements or abnormal posturing of the limbs or other parts of the body.
- Gait disorder. Trouble walking, including “shuffling,” and falls.
- Tremor. Shakiness, most often seen in the hands, but possible elsewhere in the body.
- Myoclonus. Quick, involuntary jerking of a muscle or group of muscles.
- Clumsiness. Dropping things or having trouble holding and working with smaller objects such as buttons, earrings, jewelry clasps, or eyeglass screws.
- Apraxia. Inability to perform a familiar movement on command, even though the command is understood, there is a willingness to perform the movement, and there is the capacity to move; in other words, the person cannot simply execute the movement. Apraxia can cause an inability to do common activities, such as using eating utensils or brushing teeth.
- Neuromuscular weakness. Severe muscle weakness, cramps, and flickering/twitching.
FTD patients often present two seemingly opposite behavioral profiles in the early and middle stages of the disease. Some people appear restless and lack inhibitions. Others can seem emotionally blunted and apathetic. These differences fade during late stages of the disease.
As the condition advances, so do the effects. Late-stage FTD symptoms include:
- A gradual reduction in speech, culminating in mutism.
- Inability to respond to verbal commands with motor responses.
- Hyperoral tendencies and behaviors.
- Swallowing difficulties that can cause coughing, choking, or aspiration.
- Falls or immobility due to rigidity or akinesia (loss of muscle movement).
What are the differences between FTD and other types of dementia?
FTD differs from Alzheimer’s disease and other types of dementia in several ways:
- Most people with FTD are diagnosed between the ages of 40 and 65. Alzheimer’s disease usually occurs in people older than 65 and grows more common with increasing age. There is early-onset Alzheimer’s disease, but only 1 in every 20 people with Alzheimer’s disease are under 65.
- Memory loss is a prominent early symptom of Alzheimer’s disease. Memory loss is usually not present in early FTD, but patients with advanced FTD can have memory loss.
- Behavior changes are a prominent early symptom of FTD. Behavior changes are usually not a major early symptom of Alzheimer’s disease, but they can occur with disease progression, becoming more common in late stages.
- Even in the late stages of the disease, FTD patients retain visual-spatial orientation, and they locate and navigate their surroundings accurately. Problems with spatial orientation are more common in Alzheimer’s disease.
- Although people with Alzheimer’s disease can have trouble remembering words or names, they usually have less difficulty producing intelligible speech, comprehending the speech of others, or reading than people with FTD.
- Life expectancy is slightly longer for people with FTD.
How is FTD diagnosed?
Diagnosis of FTD is based on evaluation by a doctor familiar with FTD and other dementias. The diagnostic evaluation usually includes a detailed assessment of symptoms, neurological exam, cognitive testing (also known as neuropsychological testing), blood testing, and brain scans or imaging.
Computed tomography (CT) scans and magnetic resonance imaging (MRI) reveal atrophy in the frontal and temporal regions of the brain. Positron emission tomography (PET) and single-photon emission tomography (SPECT scans) may reveal dysfunction of the frontal lobes, such as decreased metabolism or blood flow.
Blood testing is frequently done to evaluate for common, treatable conditions (e.g., nutritional deficiencies, thyroid function changes, chronic infections) that can cause similar symptoms to FTD.
Lumbar puncture and spinal fluid testing are sometimes recommended to rule-out Alzheimer’s disease, which can sometimes cause symptoms that mimic FTD.
Neuropsychological testing helps measure performance in different areas of cognitive function (memory, learning, language, visual-spatial abilities, executive function, and behavior), which could be helpful for diagnosis and measuring disease severity. Neuropsychological testing of a person with FTD could show:
- Decreased mental effort and concern. Some people impulsively respond to test items or quickly abandon participation in tests. Other people may be slow, inert, and persistent.
- Conversation is stilted. Verbal responses can be short and lack detail. Some people make mechanical, repetitive remarks, echo words spoken by others, or repeat responses.
- Apathetic patients may speak with a weak or flat voice. Patterns of speaking can be halting or awkward.
- Overactive patients may have uninhibited and uncensored speech. If you speak to them, they may respond inappropriately (or not at all). It’s not that they don’t understand – they aren’t paying attention.
- Some people have changes in their physical speaking ability, such as slurred speech or difficulty making accurate movements when speaking.
- Some people lose their grammatical processing or the meaning of words, which can impact speaking and comprehension.
- Visual-spatial skills remain normal, except for those compromised by behavioral changes.
- Memory is usually normal but may be involved later in the disease. Difficulties with attention, concentration, and mental effort, which commonly occur in FTD, can sometimes mimic short-term memory difficulties.
- Executive function (attention, concentration, verbal and design fluency, planning, judgment, problem solving, response inhibition) and abstract thinking are impaired.
Researchers are studying ways to diagnose frontotemporal disorders earlier and more accurately. They are developing biomarkers (new blood tests, spinal fluid tests, and advanced brain imaging) to improve diagnosis, measure rate of disease progression, and measure efficacy of treatment. Researchers are also expanding neuropsychological testing to improve assessment of cognitive and behavioral changes in people with FTD.
What’s the prognosis?
The length of FTD varies, with some patients declining rapidly over two to three years and others showing slowly progressive changes over a decade. Studies have shown that persons with FTD live with the disease for an average of eight years, ranging from two years to 17 years.
How is FTD treated?
No medications are known currently to treat, prevent, or slow the advancement of FTD. Serotonin-boosting medications may alleviate some behaviors.
Non-pharmacological behavioral management strategies, such as redirection or distraction, can also be used to direct behavior in a positive way. Ask the physician about therapies – medicinal, physical, occupational, speech/language, swallowing, or alternative / complementary – that they would recommend to help the person you care for.
Researchers are actively searching for new treatments and running clinical trials to test the safety and effectiveness of these treatments. If interested in clinical trial participation, please discuss this with the physician. They can connect you to resources to learn more about trial participation and help you decide if taking part in a trial is right for the person you care for.
How about the caregiver?
It’s vital for you, as the caregiver, to have breaks and time off from constant caregiving demands. Self care is essential for your own mental and physical health, and it supports your ability to care for your friend or family member when they need you.
- Start with the basics: Stay hydrated, make healthy eating choices, prioritize getting enough sleep, and ensure you are getting your regular health screenings and check-ups.
- Plan your breaks and time away from caregiving. Respite care (care of your friend or family member during your time away) can be arranged with the help of a friend or family member – you may want to consider the support of a home health worker.
- Ask for help. Some organizations even have programs to support respite care to ensure the primary caregiver gets time away. The Association for Frontotemporal Degeneration (AFTD) offers several types of grants – one of which helps cover the costs of short-term respite care for full-time, unpaid caregivers. HFC has a care grant program to cover the cost of short-term professional, in-home respite care.
- For more information, see Family Caregiver Alliance’s Fact Sheet, Taking Care of YOU: Self-Care for Family Caregivers (https://www.caregiver.org/resource/taking-care-you-self-care-family-caregivers/)
This is overwhelming. Is help available? Where do we start?
Family Caregiver Alliance
National Center on Caregiving
(415) 434-3388 | (800) 445-8106
FCA CareNav: https://fca.cacrc.org/login
Family Caregiver Services by State: https://www.caregiver.org/connecting-caregivers/services-by-state/
Family Caregiver Alliance (FCA) seeks to improve the quality of life for caregivers through education, services, research, and advocacy. Through its National Center on Caregiving, FCA offers information on current social, public policy, and caregiving issues and provides assistance in the development of public and private programs for caregivers. For residents of the greater San Francisco Bay Area, FCA provides direct family support services for caregivers of those with Alzheimer’s disease, stroke, ALS, head injury, Parkinson’s, and other debilitating brain disorders that strike adults.
Other Organizations and Links
The Association for Frontotemporal Degeneration
- FTD Disease Overview
- FTD Frequently Asked Questions
- Glossary of FTD Terms
- FTD and Clinical Trials
National Eldercare Locator
Caregiver Action Network
National Institute on Aging: Clinical Trials and Older Adults Booklet
Family Caregiver Alliance curated the information in this fact sheet, and David Solemani-Meigooni, MD, of UCSF Memory and Aging Center, provided a medical review. Sources for this information include the Association for Frontotemporal Degeneration (www.theaftd.org), Alzheimer’s Association (www.alz.org), NIH’s National Institute of Neurological Disorders and Stroke, Patient and Caregiver Education ( https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Hope-Through-Research/Frontotemporal-Disorders ). Edited for readability and user experience by Trish Doherty (http://trishdoherty.net). Revised June 2022. © Family Caregiver Alliance. All rights reserved.