Dementias’ Rarer Breed: Frontotemporal Dementia

Dementias’ Rarer Breed: Frontotemporal Dementia

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Suspecting dementia can be scary. So scary, that sometimes we become ostriches. (That whole head-in-sand thing.)

But getting an accurate diagnosis of what type it might be—if it’s dementia at all? That’s important.

Sooner rather than later.

Because dementia and memory loss aren’t a natural part of growing older. And while there aren’t currently cures if it is dementia, there may be help for some of the symptoms.

Identifying the Usual Suspects: Dementia Types from Frequent to Not So…

I believe one of the saddest things about dementia is not only losing the memories of a life already lived…
But as the disease progresses, the missed opportunities to make new ones.” (unknown)

When we talk about dementia, we often think of Alzheimer’s. Makes sense. It is the most common dementia.

Next most common is either Lewy Body Dementia (DLB) or Vascular, depending on what characteristics we’re comparing.

Then there are the dementias that evolve from other diseases, like Huntington’s and Parkinson’s.

But one dementia is rarely talked about.

That’d be Frontotemporal Dementia.

So what is it?

Frontotemporal Dementia (FTD)— When They’re Just “Different”

“The disease might hide the person underneath,
but there’s still a person in there who needs your love and attention.” (Jamie Calandriello)

With Frontotemporal Dementia, it’s all about the behavior. Memory loss only shows up late in the game. Family members complain that Dad is acting “odd,” “socially inappropriate,” even “schizoid.”

Unfortunately, these complaints happen so much that this dementia frequently gets misdiagnosed as a psychiatric problem.

But it’s far from it.

When Do Frontotemporal Dementia Symptoms Start?

The reason FTD tends to be rare in seniors is because it has a much younger onset than other dementias, with symptoms often popping up between ages 40 and 65—sometimes even in their 20s and 30s.

In fact, while it’s considered rare, it’s still one of the most common dementias for younger or midlife adults (anyone under 65), second only to early-onset Alzheimer’s. One keynote speech called it “the most common disease no one’s ever heard of.”

The numbers are a little hazy, but there may be 50-60,000 Americans with the disease.

Why is it hard to know?

Because of that tendency to confuse it with a mental (psychological) illness—or even midlife crisis.

What Are the Symptoms of Frontotemporal Dementia?

While “Dementia” is really an umbrella term for many different types, with Frontotemporal Dementia being one of them, Frontotemporal Dementia is actually an umbrella unto itself.

In other words, if Dementia is a dog, Frontotemporal Dementia is the terrier. But is it a Boston Terrier, a Bull Terrier, a Jack Russell Terrier?

Depending on where it hits the brain, it’s a different breed. And that means different symptoms.

1. Frontal Variant Frontotemporal Dementia (bvFTD):

The “b” in the initials stands for “behavioral” because the brain’s frontal lobe (the forehead area) controls conduct, judgment, empathy, foresight, …

So the symptoms, which tend to cluster and increasingly grow worse, come as no surprise:

  • Mood changes, agitation, emotional withdrawl
  • Lack of interest in daily life (apathy) and loss of empathy (no thought to other people’s feelings)
  • Low energy and motivation
  • Disinhibition and increasingly inappropriate social behavior, especially with an increased interest in sex
  • Swearing, stealing
  • Lack of judgment
  • Decline in personal hygiene
  • Crazy eating habits, often overeating, craving sweets and carbs, and even chomping on inedible objects
  • Repetitive tapping, clapping, smacking lips or other compulsive actions

bvFTD is frequently—and formally—just designated “FTD” because it’s the most common. But it’s not the only type.

2. Primary Progressive Aphasia (PPA):

Aphasia means difficulty communicating. So writing, speaking, comprehension?

They’re all targeted here.

Depending on the kind, (because yes, PPA has subcategories, too),

  • Speech may be hesitant, labored, halting, telegraphic (with the little words like “of” and “in” missing).
  • They can only speak 40 words a minute (vs the normal 140 words a healthy person can speak)
  • They struggle to put words together in a sentence. “The boy is eating chicken” might become “eat boy chicken”
  • They don’t understand what others say.
  • They lose the meaning of words.
  • They can’t name objects, just calling them “it” or “thing.”

Fascinatingly, while those with PPA may not be able to remember the word “dog” or “ball,” they may have no problem with colors, numbers, or shapes—because those aren’t stored in the same part of the brain.

3. Frontotemporal Movement Disorders:

While Frontal /Behavioral Variant and Primary Progressive Aphasia are the most frequent types of Frontotemporal Dementia, there are also several—much less common—types that affect movement.

Some resemble Parkinson’s or ALS (Lou Gehrig’s Disease).

  • Unexplained falls or balance issues
  • Loss of facial expression
  • Body stiffness or rigidity, especially in the neck and upper body or hands and arms
  • Trouble moving eyes, especially down, giving them a fixed stare
  • Tremors, muscle spasms
  • Poor coordination
  • Difficulty swallowing
  • Inappropriate laughing or crying

As Frontotemporal Dementia progresses, the possible types may blur, with one looking like the other, finally attacking memories like Alzheimer’s.

But most often? It all starts with a change in personality.

What Causes Frontotemporal Dementia?

Frontotemporal has a strong genetic link, especially compared to other dementias.

  • Somewhere between 30 to 40% of those diagnosed have another family member who also has it.
  • 10% have been diagnosed with the actual genetic mutation.
  • If your parent or sibling has it, you have a 50% chance of getting it.

But there are no other known risk factors:

  • The other 60-70% have no family history of it, and
  • It doesn’t seem to care about your gender.

However, once again, like with so many of the other dementias, there’s the similar story of renegade proteins clumping up in the nerve cells, this time not “folding right” in the frontal and temporal parts of the brain.

How and why? (Need I say it?)

They don’t know. Yet.

But the Alzheimer’s foundation is expanding their research to this and many other types of dementia to hopefully get more answers.

What Happens to the Brain in Frontotemporal Dementia?

The long and short? With FTD, the brain shrinks.

  • As different proteins for some unknown reason misbehave in the frontal or temporal lobes, they cause nerve cells in those regions to swell up and die.
  • When the nerve cells are gone, the lobes shrink.

The disease usually starts off focused, either on the frontal lobe or on one section (front, middle, or back) of the temporal lobe, explaining the varied symptoms you could see.

But over time, the whole frontal/temporal lobes may be affected, with all the symptoms piling on. By that point it’s difficult to tell the difference between types. i.e., In the end, all those terriers look the same.

In fact, it can look a lot like Alzheimers.

How Long Does Someone with Frontotemporal Dementia Survive?

Life expectancy with FTD can have a large range. Some only make it two years, some up to twelve.

But the general range seems to be about six to eight years from first symptoms. The cause of death? Often pneumonia (or some other infection) or an injury due to a fall.

And Then There’s “Mixed Dementia”—The Crossbreeds

Concentrate on where you are now, and plan on what your next step will be. Then accept that sometimes that step will not work out, will not be possible. Then look for another foothold. Tomorrow isn’t here yet, and I have survived yesterday.” (Dorothy Webb, When Goodbye Begins. Sharing Life with Dementia)

The mixed dog is all the rage now: Goldendoodles, Cockapoos, Sprocker Spaniels, the Puggles.

Problem is, because they’re blends, you don’t always know how they’re going to act.

The same is true for mixed dementias.

One out of every ten people with dementia actually has more than one kind.

  • Alzheimer’s and Vascular
  • Alzheimer’s and Lewy Body
  • Lewy Body and Vascular

To get diagnosed with both, doctors usually want clear measurable symptoms of each before they label it “mixed.” They don’t have to be equal amounts of both. One might be more extensive than the other, called “predominant.”

But if it’s all symptoms of Alzheimer’s, and then hints of the other, they keep them separate. e.g. “Alzheimer’s disease and vascular problems.”

One more reason it’s important to track symptoms.

Getting a Dementia Diagnosis

Please don’t ever leave me, do not let me go.
Because I am now so vulnerable, more scared than you can know.
So please be in my corner, now I can no longer fight.
Stay by my side throughout, and make sure I’m alright. (unknown)

As hard as dealing with dementia is, you have a lot more options when you have a name for it. Because not all dementias are created equal—or at least the same.

What will doctors use to diagnose it?

  • Symptoms
  • Neurological, cognitive and neuropsychological tests, checking balance, sensory response, reflexes, memory, judgment, problem solving, language, math skills, and other mental functions
  • Blood work
  • Brain imaging
  • Genetic testing and family history

Recent studies suggest watching someone walk can even help identify the type of dementia. (Lewy Body Dementia causes a different gait than Alzheimer’s does.)

But two extremely important areas to check?

  • Current medications to make sure it isn’t just a drug interaction.
  • UTIs (urinary tract infection)—Even if there’s no issue with going to the bathroom. (Those sneaky little infections cause all kinds of crazy mental and physical issues.)

Finding Hope in the Hard

“I believe that…Dementia takes a person’s memories. It takes their ability to communicate, to perform daily tasks, takes their freedom of choice. But it can’t take their values, their inherent personality. They hang on to those and so must we…” (unknown)

Dealing with dementia can feel very lonely. There’s a lot of loss along the way.

Don’t do it alone.

With a proper diagnosis, you can find support from those who are caring for the same type of dementia. And doctors can help you manage symptoms.

Need someone to talk to about it? Frontida cares!

Elizabeth Daghfal
• 6 min read

Elizabeth Daghfal is a writer, teacher, speaker, and community volunteer. When she isn't teaching or writing-- Who are we kidding? Her husband and five kids say she's ALWAYS teaching and writing. She has a passion to help people who are struggling and is happy to say her shoulders are drip-dry. Born and raised in the South, she now lives in Wisconsin and loves it--except for the fifteen months of winter. Read more about her at elizabethdaghfal.com.