FTD explained: Experts on brain disorder’s symptoms and treatment

Actor Bruce Willis’ family revealed his diagnosis in February

Fans and followers of Hollywood icon Bruce Willis were shocked and saddened by the news of the actor’s diagnosis with frontotemporal dementia, or FTD, a rare form of dementia.

The family shared the diagnosis with fans to help raise awareness of this condition, releasing this statement about Willis’ condition to the Association for Frontotemporal Degeneration:

“Since we announced Bruce’s diagnosis of aphasia in spring 2022, Bruce’s condition has progressed and we now have a more specific diagnosis: frontotemporal dementia (known as FTD),” the statement said in part. “Unfortunately, challenges with communication are just one symptom of the disease Bruce faces. While this is painful, it is a relief to finally have a clear diagnosis. FTD is a cruel disease that many of us have never heard of and can strike anyone.”

Those who have never heard of this condition may be wondering what it is — what are the symptoms, causes and treatments? What do you do if you or a family member is diagnosed with FTD?

What is FTD and how rare is it?

“FTD is actually a group of brain disorders that cause progressive degeneration of the neurons in the brain and, as the name suggests, affects primarily the frontal lobe, located behind the forehead, and temporal areas, located behind the ears on each side, of the brain,” said Allison B. Reiss, M.D., associate professor in the Department of Foundations of Medicine at NYU Long Island School of Medicine, and the Department of Medicine. She is also a member of the Alzheimer’s Foundation of America’s Medical, Scientific, and Memory Screening Advisory Board.

“It is irreversible and has a relatively young age of onset, most diagnosed between the ages of 45 and 64 years.”

FTD is rare with about 50,000 to 60,000 people in the U.S. having been diagnosed with an FTD, according to Reiss.

What makes FTD different from other forms of dementia?

“It occurs in younger persons; memory may remain intact until late in the disease while memory impairment is often seen early in Alzheimer’s disease. In FTD, there is no accumulation of amyloid protein, which is a key feature of Alzheimer’s disease,” said Reiss.

She stressed the importance of determining the correct diagnosis with any type of dementia, but especially with FTD, because “acetylcholinesterase inhibitors often used in patients with Alzheimer’s can lead to worsening of symptoms in those with FTD.”

“Sometimes FTD and Alzheimer’s look similar because they share areas of the brain that are affected. There is even a frontal or behavioral variant of Alzheimer’s Disease. Sometimes it can be hard to tell the difference unless you see the brain pathology or markers for it,” said Marc Haut, Ph.D., Director of the Memory Health Clinic at WVU Rockefeller Neuroscience Institute.

How is FTD diagnosed?

Definitively diagnosing FTD presents some significant challenges.

“Since it overlaps in symptoms with a number of other disorders, especially early on, the diagnosis is difficult,” Reiss said. “The patient may be subjected to a lot of neurologic and neuropsychiatric testing: The symptoms along with brain imaging and eliminating other causes of the changes in behavior — stroke, infection, head trauma — can eventually lead to the diagnosis. There is no specific biomarker to give us a diagnosis. MRI may reveal that the brain has undergone shrinkage in the key areas, and this is a good indication. (A positron emission tomography) scan showing amyloid would make FTD less likely and Alzheimer’s disease more likely.”

Is there any treatment or cure for FTD currently?

“Unfortunately, no. There is no cure for FTD, and we cannot slow its progress. Although no medications are effective in changing the course of FTD, many clinicians prescribe symptomatic treatments to minimize behavioral disturbances as necessary,” said Reiss. “Selective serotonin reuptake inhibitors (SSRIs) may help. Behavior modification techniques and, if necessary, antipsychotic medications can help.”

What is the typical prognosis upon diagnosis?

The outlook for FTD is currently not very promising.

“Life expectancy is about 8 to 10 years from diagnosis. FTD and Alzheimer’s all end similarly with language and behavior profoundly affected and memory dramatically reduced as well. 24-hour care may be necessary. Death may occur as a result of infections such as pneumonia,” said Reiss.

What are the symptoms of FTD?

According to Reiss, there is a spectrum of symptoms with FTD, in three different types or categories:

  1. Behavioral variant FTD, bvFTD, which is the most common: marked changes in behavior, deterioration of personality, executive, and social cognition. Social disinhibition, apathy, reduced sympathy and empathy, poor judgment, altered food preferences, and repetitive behavior. Relatively fewer memory problems. There may be emotional outbursts, poor manners, and excessive familiarity with strangers.
  2. Semantic variant, also known as semantic variant primary progressive aphasia. Deficits in naming and comprehension difficulties. Difficulty with language and isolated language deficits in the early stages of the disease. Over time, people lose the meaning of words and lose the ability to remember what a familiar object is or how to use it. They may substitute close words or replace a word they have lost with “that thing” or “the you know.” People with svPPA go on to show at least some of the behavioral problems seen in the behavioral variant of FTD. Speech therapy may help for a time. The use of numbers, colors and shapes can be preserved longer than words. Motor abilities are retained. Degeneration of bilateral, ventral and anterior temporal lobes occurs. Temporal lobes are used for memory, language, emotions, senses and visual recognition, according to Cleveland Clinic.
  3. Non-fluent aphasic variant. Takes a lot of effort to speak. Speech is non-fluent, and grammar is poor with impaired comprehension of complex sentences. There is halting speech with sound errors.

What causes FTD?

“The cause is unknown, except in some rare inherited cases related to specific gene mutations,” Reiss said. “More research is needed.”

What should you do if an immediate family member gets diagnosed with FTD? What are the next steps to prepare to deal with this illness?

Cloud Q. Conrad, Certified Dementia Caregiver, Trainer and Mentor, and author of “The Dementia Field Guide,” provides the following advice for families facing an FTD diagnosis:

Educate yourself. Once loved ones understand how dementia changes the brain, and where FTD concentrates in the brain, it will be easier to recognize the symptoms as a result of the pathology, rather than personality driven. Also, it’s easier to problem-solve through symptom escalations.

Join a support group. Visit alz.org to search for ones available in your area.

Consult with family attorneys and financial planners to learn about the process to establish financial and medical powers of attorney and put these and advance care planning documents in place.

Share the diagnosis with loved ones and begin to create a care plan. At first, the person can live and function nearly or fully independently. But no one person can be the sole caregiver as dementia progresses. Dementia caregiving disrupts the lives of caregivers — if possible, it’s healthiest for all to have some sort of shared responsibility for the daily care of a person with dementia. This is true both if the person will age in place or eventually move to an assisted living facility with a memory care unit.

More research is needed to provide hope and answers

“We need more research so that when physicians give this diagnosis they can give some hope,” said Reiss. “We have so many new cutting-edge technologies — I am using them in my Alzheimer’s research — that we need to apply to FTD. I absolutely believe that we can make progress if we put in the resources, and I think that the new awareness of FTD brought about by the Willis family will help to make this happen. I appreciate their courage in facing this adversity and the way that they are using their public platform for such a good purpose.”