Jul 16, 2014

Frontotemporal Dementia: Does your loved one really have this instead of Alzheimer’s?

Frontotemporal dementia (FTD) is a cluster of progressive diseases that affect the regions of the brain that control personality, behavior, language, and decision-making.

By Carole B. Larkin
Alzheimer's Reading Room

Are you sure its Alzheimer's? Did you get a "fully baked" diagnosis?


You may think you loved one has Alzheimer's when it is something else. See Dementia and Depression -- How to get a "Fully Baked" Diagnosis.

It is possible that your loved one may have another type of dementia altogether. These dementias can and do coexist with Alzheimer’s disease, resulting in something neurologists called “Mixed Dementia”, but for purposes of this article, I’m going to treat them as if they existed alone in a person’s brain.

Recently, I went to a lecture by two leading local geriatric psychiatrists/neurologists (yes they are both!) and a leading local neuropsychologist on three dementias that are not as common as Alzheimer’s, but are more common than you would believe. Each doctor lectured on one of the diseases.

Previously I wrote, Vascular Dementia: does your loved one really have this instead of Alzheimer’s?

This is the second article in a series of three.

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Dr. Kyle Womack of the University of Texas Southwestern Medical School and of the Alzheimer’s Disease Center’s Research Department presented the following information on Frontotemporal Dementia (FTD). FTD is one of his specialties and he conducts research on the diseases for the National Institute of Health, among other funding sources.

I said diseases because in truth, FTD is a number of diseases, not just one disease. Dr. Womack called them behavior syndromes because most of the symptoms shown affect our behavior as opposed to affecting our memory.

The part of our brain right behind our forehead is called the Frontal lobe and the Temporal lobes (one on each side of our head) are located roughly between our temples and our ears.

It is generally thought that Alzheimer’s starts on the sides of our heads (in the Hippocampus area) and moves forward in the brain to the frontal lobe, whereas FTD starts in the front of our head (the Frontal Lobe) and moves backwards towards the middle of the head. However, scientists and researchers are not all in agreement on this point. They are all still learning about these diseases.

Dr. Womack said that the FTD diseases come from misfolded proteins in our brain cells. Yes, we have proteins in our brain cells!

To me, the proteins look like strands of uncooked spaghetti. If you were making spaghetti and broke the strand in the wrong place (like near the end, not in the middle) when putting it in the pot of boiling water, the end result would look weird, and be hard to eat because of all the different lengths of spaghetti strands. Well, that’s how FTD works in our brains.

The proteins get folded and broken in the wrong places, and cause problems inside the brain cell (called a neuron) messing up its functioning and eventually killing the cell. Dr. Womack says the different diseases/symptoms depend on which proteins get ruined and in which part of the Frontal or Temporal lobes it happens.

Dr. Womack classifies types of FTD into 2 broad categories. The first category consists of different kinds of difficulties with language, generally called aphasia  in doctorspeak. There are different types of aphasia:

  • Some affect speech, like knowing what a word means (a word you used to know) eventually progressing to not knowing what very common words mean; words like cup, bowl, fork, etc. Some affect reading, not knowing what a word means when you read it (a word you used to know). Some affect both speech and reading. This is called loss of object knowledge
  • Some affect the fluency of speech, like slow, halting and very effortful speech. They may understand the word but no longer understand what sentences mean whether they speak them or read them. So they may be referring to the correct object (like a cup) but the sentence they use the word cup in makes no sense.
  • All aphasias affect understanding what a person means when they communicate, either the person with the aphasia or you. Not understanding what a person means naturally causes frustration, which can quickly grow to anger or tears, and greatly affect their (or your) behavior
The other broad category Dr. Womack refers to is behavior with or without problems with language. This is the most common category of FTD, he states. 

Behaviors like:
  • Impulsiveness in word or deed. For example making offensive remarks or gestures to you or others, and having no idea that they are offending anyone with what they say or do.
  • Social disinhibition, like all of a sudden taking off their clothes, or pulling down their pants and urinating on the floor, in public.
  • Over familiarity with strangers, like going up to strangers and divulging family secrets or over trusting strangers, not having a clue that some strangers could harm or take advantage of them.
  • Lack of empathy for anyone, like not really caring if something they’ve said or done has hurt or embarrassed you or anyone else, for that matter.
  • Apathy or distance in their relationship with you, where there had been closeness before their disease, such as not returning kisses, or saying that they love you back, when you tell them “I love you”.
  • Obsessive compulsive behavior, like ritualistic behavior, meaning having to do something several times before they can go on to the next thing. In the TV series Monk, his behavior was classic OC behavior.
  • Extremely resistant to changes in schedule or in plans. Way above and beyond what is normal, like refusing to go to a needed doctor’s appointment rather than being annoyed or put out about having to go to it.
  • Eating beyond being satisfied or full, like constantly eating until they vomit. Or being fixated on certain foods to the exclusion of all others. (especially sweets)
  • There are lots of other behaviors as well. The point is that those behaviors are well out of the range generally condoned by society. Care partners are mortified and angry. After all, since childhood we’ve been taught how to behave in society, and here is a loved one doing anything except what we’ve been taught as proper and not caring one bit about it.
FTD usually happens to a person earlier in life, than late onset Alzheimer’s. FTD can show up in people in the age range of 30 to 80 years old, but the average time it shows up is at 58 years old.

Recall that early onset Alzheimer’s, showing up from ages 30 to 65 is also an inherited disease.

What can be done about FTD? 

Unfortunately there is currently no cure for it and actually no approved therapies to moderate its effects on a person. That said, Dr. Womack said that sometimes antidepressants, specifically the SSRI types (that work on serotonin), meaning Celexa, Lexapro, Prozac, Paxil or Zoloft among others can help moderate the behavior in some people.

Some doctors like to use antipsychotics like Seroquel and other similar drugs to try to moderate the behaviors, but the side effects and risks to the person’s overall health are great, and can even at times exacerbate the bad behaviors, as well as raise the risk for strokes and heart attacks.

Additionally, too much of these types of medicines, can make them “zombies”.

The FDA (Food and Drug Administration) has put a “black Box Warning” on using these drugs for dementia patients, meaning don’t use them, but still some doctors do.

Dr. Womack says, don’t bother using any of the Alzheimer’s drugs on FTD, because the proteins that are damaged in FTD are not the proteins that the Alzheimer’s drugs are designed to help. Basically all you are doing is wasting your money.

So what is research pursuing in relation to FTD? Dr. Womack knows, because he is one of those researchers. He says the future for FTD research is in Genetics because FTD are really inherited diseases.

 Researchers have already identified several chromosomes in our DNA chain where the proteins are misfolded (broken) and are investigating other chromosomes as well. This research is new, much of it occurring within the last 5 years, so we know that we are still in the beginning stages of knowing all about these diseases. Still, it’s a start.

How to help care partners currently coping with a loved one with one of the diseases?

Here are a few tips;
  • When you listen to what they say, do not take it personally. The ugliness has nothing to do with you. They really can’t help it. I mean it!
  • Don’t freak out if they are hallucinating. It’s normal. If the hallucination isn’t disturbing them, then just leave it alone, or at best try to distract them with something else to look at or do. If they are freaking out move them to another location in the house, or wherever you are at the moment. Sometimes that’s enough to send the hallucination away. Then make an appointment for them with a psychiatrist. They are the only doctors that know enough about the type of drugs needed to deal with hallucinations to prescribe one and not harm your loved one.
  • If they are descending into what I call “word salad”  ask their doctor to prescribe speech therapy for them. Sometimes that helps them for awhile.
  • Consider little cards to pass out saying “This person has Frontal Temporal Dementia. It makes them act in unusual ways. Do not approach them while they are acting strange. I will handle it.” Or something to that effect, in your words. Trust me; it will save you many embarrassing moments.
  • There are support groups live and online for FTD.  Search Google using these keywords -- FTD Support Groups -- andyou will find them! Use them. They are very helpful, along with the organizations that sponsor them
Carole Larkin
Carole Larkin MA, CMC, CAEd, DCP, QDCS, EICS is an expert in Alzheimer’s and related Dementia care. She is a Certified Geriatric Care Manager who specializes in helping families with Alzheimer’s and related dementia issues. Carole can consults with families via telephone nationwide on problems related to dementia. Her company, ThirdAge Services LLC, is located in Dallas, TX.

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Original content Bob DeMarco, the Alzheimer's Reading Room