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Part 1: Understanding frontotemporal dementia (FTD)

Written By: Vicki Powers, UT Physicians | Updated: August 11, 2023
Nurse checking up on senior man

Frontotemporal dementia (FTD) is about 20 years behind Alzheimer's disease in trials and physician understanding. Spreading the word is essential so people can recognize signs.

Alzheimer’s disease is a familiar diagnosis to those who have loved ones experiencing its devastating effects. But frontotemporal dementia (FTD) is not as understood, since physicians have only given it a name in the last 30 years as they learn more. It’s challenging to recognize the disorder, even by health care professionals. Spreading the word about FTD is essential to give people resources and language around symptoms that often mimic other conditions, such as psychiatric problems, Alzheimer’s disease, or even a midlife crisis.

What is it?

FTD is aptly named because it involves the frontal lobes and temporal lobes of the brain. Portions of these lobes shrink, and the cause is usually unknown. FTD affects people differently, based on which part of the brain is affected. For some, there is more shrinkage in the frontal lobes and for others, in the temporal lobes. The similarity is how it slowly progresses over 8 to 10 years.

Paul E. Schulz, MD
Paul E. Schulz, MD

The three biggest ways that FTD presents itself relate to language, personality, and judgment, according to Paul E. Schulz, MD, neurologist with UTHealth Houston Neurosciences and professor and director of the Neurocognitive Disorders Center with McGovern Medical School at UTHealth Houston. Some with FTD might have difficulty coming up with words, knowing word meanings, and eventually losing their ability to speak. Others will have extreme changes in behavior and appear angry due to loss of inhibition. Lack of judgment is another example regarding a change in behavior out of character for that person, such as spending money frivolously.

“It’s an oddity, because it’s defined by the part of the brain involved,” said Schulz, the Rick McCord Professor in Neurology and Umphrey Family Professor in Neurodegenerative Diseases at McGovern Medical School. “It’s not like Alzheimer’s, where there’s a specific pathology in the brain. If you do an autopsy, you’ll see plaque and tangles.”

This aspect makes FTD more difficult to pinpoint and understand. In the recent diagnosis of actor Bruce Willis, for example, the family first announced he had aphasia. Months later, they provided a more detailed diagnosis of FTD, as a result of more information.

Signs and symptoms

Big personality change is often the presenting symptom of FTD, as is the case with one of Schulz’s new FTD patients in his 70s. The family initially related it to an accident, which didn’t help, he said.

“The problem with personality changes is he’s got all the thinking skills, but none of the judgment skills, and he’ll get bent out of shape with his family members,” Schulz said. “It’s very different from Alzheimer’s, where their personality is the same. They just can’t remember what you told them 10 minutes before.”

Schulz describes this patient’s brain scan where the outer part of the brain — the area where thinking occurs — may be intact in terms of the number of brain cells, but the front of the brain has very little activity. Individuals with FTD, in the early stages, can still function by finding their way around, remembering things, and knowing who they are. The problem arises when they need to make a judgment about financial things or are challenged with interpersonal or emotional situations.

“This means that all the things you do in the frontal lobe — personality-wise, filter-wise, judgment-wise — would not be working,” Schulz said. “The frontal lobe is the brake pedal for your emotions. So, if something comes up and this patient gets angry about it, his emotions get ratcheted way up because he doesn’t have that brake pedal. He starts yelling at his wife, and she’s ready to divorce him.”

Part of Schulz’s role, he believes, is helping families understand this behavior is not the person. It’s not something he’s doing on purpose. He has a medical condition.

“I’m glad if I see someone before they’re divorced, because then I can often explain it,” Schulz said. “People are a lot more forgiving if their spouse has a medical condition.”

Because it doesn’t look like Alzheimer’s, most people think there is nothing wrong, Schulz explained. They relate it to midlife crisis or being belligerent — no filter, short fuse.

Importance of understanding aphasia

Language is the second most common way patients present with FTD.

Aphasia describes a language problem, but Schulz said there are many varieties, because language is spread out over the left side of the brain.

Primary progressive aphasia localizes to the frontal lobe. Individuals experience progressive problems, mostly with connecting words, such as adjectives, adverbs, and prepositions. Schulz shared an example: The patient can say “microphone desk,” but can’t describe phrases, such as “the microphone is on the desk” or “under the desk.”

“Recognizing primary progressive aphasia is important clinically, because that gives me the idea that it’s often caused by FTD pathology,” Schulz said.

If it turns out it’s Alzheimer’s-related and it’s early, Schulz can put the patient in an Alzheimer’s trial, because there are things that are testable with positive outcome.

“If it’s FTD, I’m going to work on education and that kind of direction,” Schulz said. “I don’t have too many trials for that yet, because we just are not that far along.”

Explore the signs

The most important thing people can do if they experience these types of symptoms, according to Schulz, is to recognize their concerns and visit their doctor. The age of onset for FTD can begin in people’s 20s and as high as their 80s, but most patients are in their 50s.

“Very few people go to their doctor, because there really are no symptoms for FTD that are recognized like with disorders such as Parkinson’s,” Schulz said. “The MRI is normal, the cognitive quiz is normal, and yet you can still have FTD, because it’s not really a cognitive disorder, per se.”

He also emphasizes the importance of getting a second opinion, because not everyone has seen a case of FTD yet, even neurologists. As a resident in the 1980s, Schulz had never knowingly seen a case of it. He actually had, but didn’t realize it was something other than Alzheimer’s.

“Back then, results were either normal, hardening of the arteries, or Alzheimer’s. That was it,” Schulz said.

After following these symptoms until someone died, the neuropathologist would confirm say it wasn’t Alzheimer’s, but something else.

Now they’ve figured out it’s not one disease at all, but many diseases. Schulz has 18 different genes he tests, meaning 18 different pathologies, all related only in that they involve the same part of the brain. It looks similar to other diseases, because they involve the same part of the brain and progress in the same 8 to 10 years — except ALS, which is quicker.

“FTD is 20 years behind Alzheimer’s regarding trials, so it’s a tougher disorder now,” Schulz said. “We don’t have ways of affecting the underlying process, but I would argue we do make quality of life a lot better. Part of that is with medication.”

Watch for the next installment about frontotemporal dementia — factors around heredity, messaging to families learning of a new FTD diagnosis, and best ways to support the patient with FTD as well as the caregivers.

As the clinical practice of McGovern Medical School at UTHealth Houston, UT Physicians has locations across the Greater Houston area to serve the community. To schedule an appointment, call 888-4UT-DOCS.