Normally, when we think of dementia, we mostly think of it as an illness that affects the elderly. While that is generally true, there is one form of dementia that can strike at a rather young age. Fronto-Temporal Dementia, or FTD, is one of the leading forms of dementia after Alzheimer’s Disease, Vascular Dementia, and Lewy-Body Dementia.
Although comparatively less prevant than Alzheimer’s, Parkinson’s, or other neurodegerative diseases, which usually affects individuals who are of a more advanced age, for FTD, age is just a number. It can strike when people are as young as in their mid 20s. As such, FTD may end up having a larger impact on the patients life and those around them over the course of the illness.
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QUICK LINKS - TOPICS COVERED:
- WHAT IS FRONTO-TEMPORAL DEMENTIA?
- WHO GETS FTD?
- WHAT ARE THE CAUSES OF FTD?
- STAGES AND SYMPTOMS OF FTD
- DIAGNOSING THE ILLNESS
- TREATMENTS FOR FTD
- CHALLENGES OF CARING FOR A FTD PATIENT
- CARE ARRANGEMENTS FOR FTD PATIENTS
- RESEARCH AND CLINICAL TRIALS FOR FTD
- RESOURCES & SUPPORT
- IN THE NEWS - FRONTO-TEMPORAL DEMENTIA
WHAT IS FRONTO-TEMPORAL DEMENTIA?
FTD (also known as Pick’s Disease) is a term used to describe a form of dementia that causes neuronal death and progressive damage to the frontal and temporal lobes of the brain. It is characterized by behavioral and personality changes along with a decline in the ability to empathize, learn, reason, make judgments, communicate, and eventually carry out daily activities.
The frontal lobes of the human brain, which is located directly behind our forehead is responsible for controlling behavior, emotion, attention, judgment, planning, and self-control. Damage to this area leads to reduced intellectual abilities and changes in personality, emotion, and behavior. The temporal lobes of the human brain on the other hand are responsible for processing language, especially what we hear and see. Damage to these areas primarily leads to difficulty understanding or expressing language.
WHO GETS FTD?
Studies show that the onset of FTD usuall occurs in people in their 40s to 60s. About 60% of cases occur within this age bracked. However, there have been instances where individuals as young as those in their early to mid 20s have been diagnosed. Other studies estimate that less than 3.5 people in every 100,000 may have FTD as it is less common compared to Alzheimer’s and other common forms of dementia. However, researchers suspect that it might be heavily under-diagnosed as its symptoms can often be mistaken for other illnesses.
WHAT ARE THE CAUSES OF FTD?
To date, the exact cause of FTD is unknown. However, researchers have identified abnormal protein structures, known as Pick bodies, that accumulate in the frontal and temporal lobes of the brain to possibly cause nerve cell death and followed be degeneration. As the disease progresses, the shrinking of the frontal and temporal lobes is also present, leading to behavioral and personality changes as well as decline in language comprehension capabilities.
There are many studies researching the possibility of hereditary components to FTD. While evidence to support the theory is still largely ambivalent, the possibility remains as some types of dementias do end up being genetically passed down.
In the News: Causes of Fronto-temporal Dementia
In the News: Prevention
STAGES AND SYMPTOMS OF FTD
The progress of FTD can vary dramatically from person to person. There are both cases of accelerated progression of just a couple of years as well as rates of much slower progression where a patient deteriorates over more than a decade. But on average the symptoms progress and become more pronounced over a course of 8 to 10 years.
The symptoms of FTD depend on the area of the brain that is affected. In contrast to Alzheimer’s, memory often remains unaffected until the later stages. And although during the initial stages, a person may exhibit distinct symptoms based on the area of brain that is first damaged, over time, as the dementia progresses, the symptoms converge. If the frontal lobes are affected first, changes in behavior and personality become apparent. This is called Behavioral-Variant FTD.
On the other hand, if the temporal lobes are affected first, the loss of language skills becomes obvious. This is known as Primary Progressive Aphasia. Within it, there are two types: progressive non-fluent aphasia and semantic dementia. In progressive non-fluent aphasia, the person’s speech becomes hesitant and lacks grammatical accuracy. In semantic dementia, patients lose their ability to understand or formulate words in a spoken sentence.
Stage 1: Cognitive Impairment
Stage 2: Early Stages
Often the first symptom reported by close friends or family of a patient is disinhibition, apathy, loss of sympathy or empathy for others. Social withdrawal and lack of interest in family and friends may be evident as well. While they also tend to exhibit some obvious problems with planning and organizing, the person is still very much capable of managing household tasks and independent living. One thing to be careful of is that due to impaired judgment, the patient may make financial indiscretions with negative consequence or behave inappropriately in public.
Progressive Non-Fluent Aphasia
This type of FTD leads to increasing trouble with speaking and language production. While the patient usually understands and is aware of what they would like to say back, they have trouble articulating it. Early symptoms include slowed speech and trouble speaking the words correctly. For example, if the patient were to repeat a word several times, it will likely sound slightly different each time they say it.
Patients with semantic dementia that is predominantly on the left side of the brain initially complain of a hard time coming up with the words or name for things. Instead they may replace them with terms such as ‘thingy’, ‘you know’, or other filler words. Day to day memory is still intact at this stage. On the other hand, patients with semantic dementia that is predominantly on the right side tend to exhibit a decline in empathy and awareness of others’ emotions.
Stage 3: Middle Stages
Progressive Non-Fluent Aphasia
Stage 4: Late Stages
Progressive Non-Fluent Aphasia
DIAGNOSING THE ILLNESS
When a person exhibits initial behavioral change, often the symptoms are mistaken for personality disorders such as depression or bipolar disorder. It takes a highly experienced pathologists with knowledge of FTD to diagnose the person correctly. Brain imaging scans such as an MRI can help with this. During the early stages, it will show mild atrophying in certain areas of the frontal lobe. As the dementia progresses, shrinking of the brain will increase.
Many of the symptoms of FTD can very often be explained by other illnesses or deficiencies. Therefore, highly trained physicians will first conduct multiple tests and evaluations to rule out those possibilities before looking at FTD as the cause. The challenge for them is to correctly identify the cognitive and behavioral changes.
If a close friend or family member suspects that a person has FTD, they should immediately consult their local physician. The local physician then may refer them to a neurologist, behavioral neurologist, or neuropsychologist. Usually, the following items are involved in the diagnosis of FTD:
- Extensive and detailed neurological examination and medical history
- Neuropsychological examination in order to evaluate language, behavior, memory, executive and visual-spatial functions
- Neuroimaging to determine where are the most affected brain regions, and in what extent, and which brain regions have suffered atrophy or might be experiencing a blood flow decrease. Examples of neuroimaging techniques: MRI (magnetic resonance imaging), PET (positron emission tomography), and SPECT (single photon emission computed tomography)
In the News: Detection & Diagnosis
TREATMENTS FOR FTD
As is the case with most dementias, FTD currently does not have a treatment available. There is no way to stop or reverse the symptoms. However, there are several types of medications and therapies available to help slow down the progress or decrease the severity of the symptoms such as anti-psychotics and anti-depressants.
The kinds of anti-depressants that are often prescribed to FTD patients are called selective serotonin reuptake inhibitors (SSRI). They are useful in reducing aggressive impulses, poor impulse control and cravings. Some common brands include: Prozac, Zoloft, and Paxil.
As for anti-psychotics, most are used to block the effects of dopamine, which is a chemical messenger that may be responsible for hallucinations and anxiety. Low doses of these can help to manage aggressiveness and irrational and compulsive behaviors. However, the adverse effects should be weighed in as they can lead to weight gain and difficulties in both moving and thinking. Some common brands and anti-psychotics prescribed are: Risperdal, Tegretol, and Zyprexa.
In the News: Treatment
CHALLENGES OF CARING FOR A FTD PATIENT
One of the most important things to keep in mind when taking care of an individual with FTD or any other forms of dementia or neurodegenerative disease is that you need to be extremely patient with them. Staying calm can help keep both the patient’s and their caregiver’s stress levels low.
Stage 1: Cognitive Impairment
- Find Support for the Patient and Yourself: One of the first challenges that a caregiver will face is accepting the diagnosis. This can be quite difficult for both the patient and the caregiver. Talking to a therapist can help to come to terms with the new situation. For support groups and resources, visit the resource and support section here.
- Prepare for Long-Term Care: Next, getting the patients affairs in order and preparing for long-term care is extremely important. The patient may be able to still express desires for how to handle certain financial and medical situations. Talk through the difficult issues with them and make a plan for how to deal with long-term care. Typically once a person has been diagnosed or the symptoms are more advanced, a close family member is designated Power of Attorney for Medical, Financial and Legal matters. Nail all of these aspects down and have a clear path of what will happen and how.
- Get the Right Medical Advice: Seeking a medical group that specializes in these types of conditions will be extremely beneficial as they will have many resources (social workers, support groups) that are specific to the patient and their families needs. To find a provider near you, visit the Association for Frontotemporal Dementia's page that describes the different types of Doctors available, here. Click on the International, Canadian or United States “In You Region” pages to access information on centers devoting resources to frontotemporal degeneration. You can also look out for clinical trials that may help to benefit the patient.
- Adjust the Home and Establish a Routine: The next step would be to make some modifications to the patient’s daily routine and home in order to accommodate their symptoms.
- Establishing a Routine: Routines for a person with dementia are critical. They help maintain the person as healthy as possible. A care routine will also be needed to prevent caregiver burnout. Use Calendars that can be shared such as Google Calendars, or caregiver coordination softwares such as Lotsa Helping Hands to get organized.
- Memory Loss: Initially, memory loss might be subtle and almost unnoticeable, eventually becoming very obvious. For tools to assist with Memory Loss during this stage read Memory Loss and the Early Stages.
- Driving and Coordination Issues: Driving is commonly one of the first issues FTD patients struggle to cope with. For tips on how to manage this, go here.
- Mobility Issues: The patient may suffer from affected coordination. Tips on dealing with Trip and Fall Hazards, go here.
- Frustration/Depression: Coping with the changes of dementia and feeling their mental and physical abilities deteriorate is extremely difficult and to witness as well. Be emotionally supportive and caring for the individual. Seek professional assistance for them and for yourself. There are great resources out there for this, many of which are listed here.
Stage 2: Early Stages
- Memory Loss & Speak Impairments: Memory loss and speech impairments will have progressed significantly during this time. For tools to assist with Memory Loss during this stage read Memory Loss and the Early Stages.
- Wandering: Many FTD patients wander and it can be extremely dangerous. Prepare for wandering and other Safety Concerns by reading Top 11 Concerns for Dementia with Solutions.
- Restlessness/Anxiety: Keeping the patient active with exercise and activities will help reduce anxiety during the day. Before the disease, the individual most likely had an active life full of work or social activities. Transitioning to a more limited life is difficult for patients with dementia and keeping them engaged in the community and their environment helps them cope and feel useful.
Stage 3: Middle Stages
- Poor Coordination: Tips on dealing with Trip and Fall Hazards, go here. For tips on dealing with mobility issues in the shower, go here.
- Wandering & Anxiety: Many FTD patients wander and it can be extremely dangerous. Prepare for wandering and other Safety Concerns by reading Top 11 Concerns for Dementia with Solutions.
- Sleep Issues: Dementia patients typically experience sleep disorders. Keeping up with the routine and ensuring the patient has copious amounts of exercise and stimuli during the day will help with this. For tips on preparing the bedroom for a person with FTD, go here.
- Aggression: Be patient with your loved one. Give them plenty of space when they are experiencing an aggressive spell. Identify what is triggering the aggression. Is it being told they can't do something? Or taking away food? Find the triggers and identify solutions to decrease the potential for them to exist. Consult with their physician to identify if medication may be a good solution for lessening the aggressive episodes.
- Paranoia/Hallucinations: Hallucination and delusions can occur with FTD. They may be a side effect of medications or they can be caused by deterioration to the brain. Consult with the patients physician if you notice these changes.
Stage 4: Late Stages
- Severe Memory Loss: The patient may no longer be able to remember those closest to them or their surroundings. They will need assistance with every aspect of caring for themselves but that does not mean that they are completely gone. Many patients respond positively to music and dancing. Keep around what you know they enjoy and try to look at the positives.
- Severe Mobility and Speech Issues: At this point, assistance with movements, eating, showering, toileting are needed. Keeping the person hydrated and massaging their aching muscles will be of great relief to them.
- Incontinence: Your loved one will lose the ability to go to the bathroom without assistance. Transitioning to diapers can be a challenge but after a while becomes routine. Ensuring they are clean is critical to keeping bed sores and skin conditions from becoming an issue. We at Dementia Aide have designed a onesie that helps with the issue of incontinence, reducing the caregiver burden and stress/agitation for your loved one. It zips in the back to prevent undressing and to help with transitioning to diapers. For more information - visit the Bear Hug Onesie product page:
CARE ARRANGEMENTS FOR FTD PATIENTS
In terms of care arrangements for FTD patients, there are generally two options that the patient’s friends and family can opt for: in-home care or out-home care. During the initial stages, while the patient is still able to function independently, most people may prefer to remain at home, where they are most familiar with their surroundings.
After initial diagnosis, the primary caregiver may still be able to handle the tasks of looking after the patient. But as the symptoms progress to the early and middle stages, they can opt for in-home care arrangements. There are several types available in this category:
- Companion services – to help with supervision and recreational activities.
- Personal carers – to help with various personal care tasks such as bathing, dressing, eating, and exercising.
- Homemaker services – to help with housekeeping, cooking, or shopping.
- Medical carers – to help with medical needs, physical, and/or mental therapies
Another option for patients who are still able to function relatively independently, is adult day care centers. These facilities provide a safe environment for dementia patients to pursue activities, interests, and socially interact with people. This type of care arrangement can give the patients a sense of freedom while dealing with their diagnosis. They are also likely to have counselling, health services, meal services, personal care, behavior management, therapy, and other related services available.
During the last stages of dementia, when the patient is unable to fend for themselves, the best choice often will be residential care in the form of assisted living, nursing homes, or special care units. There, they can receive specialized care and treatment to ease their situation. Each of these facilities will offer different levels of care. Selecting one will depend on the patient’s needs and progression of FTD.
RESEARCH AND CLINICAL TRIALS FOR FTD
FTD is still a rather poorly understood illness and often leads to under- or mis-diagnosis. One of the best ways to better understand FTD is to study the disease more in-depth. And to do that, participating in research and clinical trials can be very beneficial. In addition to helping to advance the understanding of FTD, patients can often benefit from the clinical trials. Below are a few types of studies that can be found:
- Treatment trials – where new and existing drugs are tested to study its effects on FTD symptoms
- Genetics studies – where scientists look into a possible genetic connection to the illness
- Diagnostic studies – where researchers seek better and improved methods of diagnosing FTD
- Prevention trials – where researchers investigate for ways to prevent the onset of FTD itself
Finding Currently Recruiting Clinical Trials and Studies
- AFTD maintains a listing of Currently Recruiting Clinical Trials and Studies, updated quarterly.
- ClinicalTrials.gov – maintained by the U.S. National Institutes of Health (NIH) of federally and privately funded clinical research, the registry provides patients, family members, and members of the public easy and free access to information on clinical studies for a wide range of diseases and conditions. A search feature lets you look specifically for studies in FTD. Enter “frontotemporal degeneration”, “frontotemporal dementia”, or search terms for the specific FTD disorders: primary progressive aphasia, progressive supranuclear palsy, cortical basal degeneration, or ALS and FTD to view a full list of all registered trials, eligibility criteria, participating medical centers and contact information for each.
- NIH Clinical Center – conducts research in a wide range of diseases, including frontotemporal dementia. The Clinical Center’s website is designed to help people learn more about clinical trials, why they matter and how to participate. In addition, health care professionals can read about evidence-based strategies for talking with patients about trials, and find audience-tested posters to help promote trials in clinics and offices.
- ARTFL Network – a consortium of academic medical centers partnered with patient support organizations conducts clinical research in sporadic and familial FTD. ARTFL is funded by the National Institutes of Health and is part of the Rare Diseases Clinical Research Network.
- LEFFTDS – conducts longitudinal studies of people in families who have inherited a pathogenic mutation in progranulin, C9ORF72, or tau. A primary goal of LEFFTDS is to identify the most robust and reliable methods to track disease progression in familial FTD so that disease-modifying therapeutic trials can be designed appropriately.
In the News: Research
RESOURCES & SUPPORT
For family members and friends who would like to educate themselves further on the details of FTD, its symptoms, treatments, and challenges, you should seek the assistance of local or national dementia association. Below are some resources to help start your research:
Association for Frontotemporal Degeneration (AFTD)
Radnor Station Building #2 Suite 320
290 King of Prussia Road
Radnor, PA 19087
National Institute on Aging (NIA)
National Institutes of Health, DHHS
31 Center Drive, Rm. 5C27 MSC 2292
Bethesda, MD 20892-2292
- Association for Frontotemporal Degeneration: Support Group Listing
- Online Forums & Chat Rooms:
- University of California, Aging and Memory Center: FTD Support Forum
- Alzheimer's Association Forum: AlzConnected
- Facebook Groups:
- Dementia Information
- Frontotemporal Dementias Info and Support
- Forget Me Not - Frontotemporal Dementia (FTD)
- The Association for Frontotemporal Degeneration (AFTD)
- Hidden Group for Young Adults: Association for Frontotemporal Degeneration (AFTD) - Young adult Facebook group for people in their 20s and 30s who have a family member or loved one with FTD. This group serves as a supportive online forum and gathering place for young adults. To join the “secret” group, please email firstname.lastname@example.org and include the email address you used to open your Facebook account. You can expect an invite to join the group within a week.
FTD Spouse Caregiver Support
An independent, online, peer support group committed exclusively to the spouses of those diagnosed with frontotemporal degeneration and early-onset dementias.
- Anonymous chat rooms for Free therapy: 7 Cups
- Informal Caregiver Connections: Sometimes AFTD can match FTD caregivers in a particular city or region through information they share when they register. It is an informal connection that can be helpful if there are no face-to-face groups available. AFTD identifies people in the network who are in a similar situation and within a reasonable distance from you. After agreement by both parties, AFTD shares a name and a contact number or email, but no other personal information. You take it from there. To request a search, please contact AFTD’s HelpLine at email@example.com or toll-free 866-507-7222. You can also register with AFTD on-line.
AFTD’s Comstock Respite and Travel Grant Program provides grants to help full-time, unpaid caregivers to arrange short-term respite. Making sure to have some time for yourself is an important aspect of support.
Lotsa Helping Hands powers free online caring communities that provide tools to organize daily life during times of medical crisis or caregiver exhaustion. They facilitate an Open Community model to connect caregivers, individuals, and families who need help with those who want to lend a helping hand.
The Well Spouse Association (WSA) is a non-profit, self-help, and volunteer-based organization whose mission is to provide peer emotional support and information to the husbands, wives and partners of the chronically ill and/or disabled. WSA offers local area support groups, mentors, respite weekends, an online forum, and more. Call WSA at 1-800-838-0879 to learn more.
Caregiving.com is a community of supportive individuals caring for a family member or friend. Whether you are caring for your parents, spouse, sibling(s), grandparent(s), or anyone you consider family, this page is dedicated to providing you with resources before, during and after caregiving. Listen now to a podcast on Managing FTD’s Challenging Behaviors here.
IN THE NEWS - FRONTO-TEMPORAL DEMENTIA:
Click the following links for News Articles on Fronto-temporal Dementia (FTD):