Frontotemporal Degeneration: A complex disease – Webinar notes

Frontotemporal Degeneration: A complex disease – Webinar notes

In late December 2019, the Alzheimer’s Foundation of America offered a webinar on frontotemporal degeneration (FTD), which includes two atypical parkinsonism disorders: corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). The speaker was Sharon Denny, Senior Director of Programs for the Association for Frontotemporal Degeneration (AFTD). She discussed the different FTD syndromes; the types of changes and complex care needs that can occur; and the impacts on the family and caregivers. We at Stanford Parkinson’s Community Outreach listened to the webinar and are sharing our notes. Our summary focuses on the two atypical parkinsonism disorders – CBD and PSP – which we felt would be of interest to some readers.

The webinar was recorded and can be viewed on YouTube.

If you have questions about the webinar, you can contact the Alzheimer’s Foundation of America via phone at 866-232-8484 or via email at

The Association for Frontotemporal Degeneration (AFTD) is the leading national organization and a global leader focused exclusively on the FTD disorders. Their website is here. They also have a Help Line: 866-507-7222.

The Brain Support Network has additional resources for those wishing to learn more about corticobasal degeneration and progressive supranuclear palsy.

Now… on to our notes from the webinar.

– Lauren

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Frontotemporal Degeneration: A complex disease with complex care needs – Webinar notes

Presented by the Alzheimer’s Foundation of America

Originally aired December 12, 2019

Summary by Lauren Stroshane, Stanford Parkinson’s Community Outreach

Sharon Denny is the Senior Director of Programs for the Association for Frontotemporal Degeneration (AFTD), where she advocates for the inclusion of persons living with FTD. She also encourages community involvement across AFTD’s volunteer, support services, education and advocacy programs. In this webinar, she spoke about the signs and symptoms of the three main FTD syndromes, described the current best approaches to care, and discussed how to get involved in research.

Frontotemporal degeneration (FTD) is the most common cause of dementia in those under the age of sixty. The age of onset can range widely, from 20 to 80 years of age; the average individual is diagnosed at age 57. About 20 people per 100,000 are affected, making this a rare group of diseases; by contrast, a 2018 study found that Parkinson’s disease affects about 572 people (over the age of 45) per 100,000. The signs and symptoms of FTD-spectrum disorders vary widely and can involve behavior, language, and movement. It is not primarily a memory disorder, though memory can sometimes be affected.

FTD is an umbrella term for a spectrum of overlapping disorders, caused by multiple different protein pathologies: tau, TDP-43, and FUS. The genetics of these disorders are complex; about half of cases are “sporadic,” meaning arising without a known genetic cause, while 15-20 percent are passed down from a parent (“dominant” inheritance).

This summary will primarily focus on the movement disorder variants of FTD: corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP).

Cognitive changes principally impact executive function: activities that require attention, such as reading books or watching movies; making and executing plans; and the ability to reason and think flexibly. Changes in behavior can also occur, more typically with the behavioral variant FTD, and can include apathy, lack of inhibition, impaired judgment, and loss of empathy.

Progressive supranuclear palsy (PSP) is a variant of FTD that is characterized by imbalance and unexplained falls; stiff, slow movements; and trouble coordinating eye movements. To learn more about PSP, read more here.

Corticobasal syndrome (CBS) is an FTD variant that causes rigid, slow, reduced movements, apraxia (inability to perform tasks or movements despite knowing how), and limb or fine motor control. To learn more about CBS, read more here.

FTD presents distinct care & support needs

FTD can have a huge impact on the family, causing a shift in roles. Many individuals have been coping with symptoms before being diagnosed and didn’t understand what was going on. Relationships are often strained or broken prior to diagnosis. Loss of employment or ability to work, often at the peak of one’s career, can substantially impact the family’s finances. Individuals are often still physically robust and active, which adds a layer of consideration to treatment decisions, and often have fewer concurrent health conditions.  

As the disease progresses, the caregiver is essential to maintain safety and quality of life for the individual with FTD. If there is a behavioral component, behaviors can be intrusive or embarrassing, and friends often pull away. There is often a sense of ambiguous loss; sometimes the changes that affect the family aren’t readily apparent to outsiders.

In 2017, a study characterized the economic burden that affects families of those with FTD in the United States, where support and services are often unavailable. Household income fell as much as 50 percent in the 12 months prior to an FTD diagnosis, and 37 percent of caregivers stopped working after their loved one was diagnosed with FTD. Due to lost income and costs of treatment and care, the study estimated an economic burden of around $120,000 a year for families of those with FTD, roughly twice the reported economic burden of Alzheimer’s disease

Care management challenges

Unfortunately, there are currently no treatments or clinical care guidelines for FTD. Many physicians and healthcare providers do not have experience with FTD; few home and community services are familiar with these diseases, and eligibility for those under 65 years often limited. There are no medications for specifically for cognitive symptoms (i.e. awareness, judgment), though some treatments may help symptomatically with movement issues.

Isolation and “care burden” are higher in FTD, and the progression of movement changes in CBS and PSP can be difficult to predict. The speaker recommended tailored approaches to care, depending on the needs and abilities of each individual.

For those working with FTD, she advised paying attention to the family’s unique experience of the illness and diagnosis journey. The diagnostic experience can shape subsequent contacts; it is important to be aware of what the family’s experience has been leading up to diagnosis. Frustration, anger, and guilt are all common experiences. Once a diagnosis has been made, families may need assistance to make sense of what they have learned – expanding the concept of dementia to include someone who is younger and mobile, and reframing relationships to meet the new normal.

Education about the disease is the best intervention to ensure a well-informed and supported person with FTD and care partner. Not only does an understanding of FTD (and related subtypes) help to improve quality of life for the individual, but an educated care partner will be better able to observe and describe symptoms to the healthcare provider. The family can also be taught the importance of research and emerging clinical trials. If available, counseling can be beneficial for the individual, the couple, and any children.

FTD can be a very isolating experience. Those who develop these diseases may not feel like they belong in support groups for individuals with PD or AD. Meeting those who understand is critical. Peer support (face to face, via phone, or online) can be immensely helpful and can be a source of creative problem-solving assistance, or sharing local resources.

Care strategies for FTD

Many with FTD still have things to offer and want to be involved; try to maximize their inclusion in activities and events. Adjust expectations, maximize engagement, and still do preferred activities – just differently! Assess the needs of the family system and try to attend to the needs of each member.

Approaches used with Alzheimer’s patients may not be beneficial for those with FTD. Non-pharmacological interventions are more effective than medications. Providing a simplified, structured environment and partnering with the family to provide a true team approach is best.

Assess for safety regularly. Are they safe to be driving? Walking around on their own?  Traffic signals? Having access to power tools or firearms?

Finding appropriate services is vital, but difficult and exhausting for families. Try to build a care team that includes:

  • An FTD specialist (usually a neurologist or neuropsychologist), a general physician, and a palliative care consult
  • OT, PT, and speech language therapies
  • Home care, creative companion care, residential options

Advocate for benefits, such as retirement and short-term or long-term disability benefits. Look into Compassionate Allowances, SSDI, and Medicare, though these all are complicated by an uncertain diagnosis early on.

Research in FTD: momentum is growing!

Biomarkers research is striving to improve diagnosis. Currently, the only way we have to be certain what pathology is present is after death, with a brain autopsy (unless an identified genetic mutation is present). If scientists can identify biomarkers for FTD, this will provide earlier diagnosis.

Drug development and clinical trials are needed for the different biological subtypes; we won’t get a medication to treat both behavioral variant FTD and CBS, for instance. Treatments will try to target those with pathology from tau, TDP43, GRN mutation carriers, and C9ORF72 mutation carriers.  Applied technologies are being developed to improve care and reduce care burden.

The ALLFTD Study is a research consortium funded by an NIH grant, taking place across 19 different sites in the US and Canada. They are performing natural history studies with cohorts of people over time to understand how the disease develops. Some people don’t have a family history; others have a known mutation. For those who have genetic factors, the study may illuminate when they start to show symptoms, and can help target treatments eventually. To learn more, visit their website.

The FTD Disorders Registry is a private, HIPAA-protected database of individuals with FTD, family members, and friends who can sign up to receive information about any important research updates and opportunities related to FTD. To learn more, visit their website.

The Association for Frontotemporal Degeneration (AFTD) is the leading national organization and a global leader focused exclusively on the FTD disorders. They provide a help line, disease education, care management information, a list of FTD support groups, and information on research. Contact information is listed at the beginning of this webinar summary, and their website is here.

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Question & Answer Session

Q: What is an example of a neuropsychological test?

A: In its fullest form, usually a battery of paper and pencil tests administered by a neuropsychologist. It often takes about 3 hours. The test results can be valuable for diagnosis but can be pretty exhausting for the participant. The test looks at different cognitive skills to see where strengths and weakness are in the person’s thinking skills. For some, it might be language skills, mental flexibility, and memory. Functional strengths and weaknesses in terms of their thinking skills are part of the evaluation.

Q: Is there an average life expectancy for someone with FTD?

A: The average is not very helpful for FTD – it is typically 6.5 to 7 years after diagnosis – but the diagnostic time frame is so variable that this isn’t necessarily a meaningful number.

Q: Can you give an example of an intervention that would be different in FTD than in AD?  

A: What are the changes in the environment that are going to reduce the disruptive behaviors? Look at the environment and see what we can change to work for that person. An additional challenge is that those with FTD often still retain memory, unlike those with AD.

Compulsive behaviors, such as eating or wandering– nothing is going to completely stop this. But lots of symptoms are triggered by visual stimuli. For instance, is someone gets focused on eating cookies, remove visual triggers. Put away the cookies out of sight!

A lot of non-pharmacologic interventions are about changing the environment or the providing additional support for the person with FTD. Those with FTD can be much more vulnerable to online scams, since they may have access to computers but impaired judgment. Try to find ways to allow them to still use the computer but limit those risks.