what is frontal temporal dementia?


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I attended a family seminar on Frontal Temporal Dementia (FTD) and Primary Progressive Aphasia (PPA) yesterday at the Mayo Clinic in Rochester, MN. It was of particular interest to me as my family carries the familial gene for FTD. I learned a lot, probably more than I really wanted to know.

Because FTD has a different presentation (onset and symptoms) than the more commonly known Alzheimer’s Dementia, I thought it might be helpful to describe it.

FTD is part of a larger cluster of neurodegenerative diseases  that includes ALS (Lou Gehrig’s Disease), PPA, and Corticobasal Syndrome, to name a few. This cluster of diseases is called Frontal Temporal Lobar Degeneration or FTLD. Diagnosis is based on a person’s symptoms, brain scans and genetic testing… and most accurately through autopsy.

Even within the FTD group there is variability with the most common being the behavioral variant frontal temporal dementia or bvFTD. I believe that is the type that my father had.

Frontal Lobes

The frontal lobes, located at the front (behind the forehead) of the brain,  are responsible for executive functioning. This includes planning and sequencing, prioritizing, multi-tasking and recognizing/correcting errors.

Imagine trying to prepare a simple meal if you have difficulty thinking through the steps necessary to put a meal on the table for your family or to fail to see errors and correct them. What happens to a cake when the eggs or liquid is forgotten… the oven never turned on… or worse, baked at too high a temperature?

illustration of parts of the brain

The frontal lobes also help with managing emotions, decision-making, and language. When the frontal lobes become damaged, persons with bvFTD are often inappropriate in social situations and lack insight about their behaviors. One of the presenters shared a story of a person with  bvFTD who walked over and took a bite out of a stranger’s sandwich when dining at a restaurant to see if he liked it before ordering his own. Disrobing in public is not uncommon.

In later stages, the person with bvFTD has difficulty forming sentences  and with overall motor functions. My dad lost his language quite early but his motor skills remained in tact until the last year or so. He could no longer recognize or use a fork or spoon and required help with the simplest of tasks.

Temporal Lobes

The temporal lobes, located  below and to the side of each frontal lobe on the left and right sides of the brain, play an important role in memory retention and a major role in language and emotions. They help people understand words – both spoken and written. Without the temporal lobes, we are unable to connect words with their meanings, recognize common objects, or recognize emotions and respond appropriately.

What might bvFTD look like?

No two persons with bvFTD look-alike and each progression is unique as it depends upon the areas of the brain affected first, environmental factors, and contributing co-factors (such as other illness).  The major behavioral symptoms of bvFTD identified in the workshop are listed as follows:

  • dramatic changes in personality
  • loss of social skills
  • socially inappropriate behaviors
  • loss of empathy
  • loss of insight
  • impulsive actions
  • compulsive and stereotyped actions
  • inflexible thinking
  • emotional flattening (inability to show emotion)
  • bizarre somatic complaints
  • excessive eating, gluttony, poor table manners
  • loss of attention to appearance and hygiene
  • roaming

My biggest take-away was the lack of insight. Persons with bvFTD have no insight into their deficits. I attended a breakout session for persons with a diagnosis of FTD or PPA. There was a gentleman, clearly well-educated based on the work that he used to do, who could not understand why they took away his license. He worked as a consultant and without a license he was unable to visit his clients. He was a large man, wearing a t-shirt that was dirty and did not cover his large belly. He was unshaven… his overall appearance was unkempt. Perhaps a classic example of someone with bvFTD?

How are the FTLD types different from Alzheimer’s Disease?

FTLD may account for 10 percent of all cases of dementia and is as common as AD among those younger than 65 years. FTLD is often misdiagnosed in AD, especially in the early onset types. Sixty percent of people with an FTLD are 45 to 64 years old.

The average life span from  diagnosis of a FTLD type is around 10 years while persons with AD are usually affected later in life with an overall slower progression.

The challenges in AD tend to revolve around the memory problems. They have difficulty with memory around events or people and may become extremely anxious as a result. Their behaviors tend to be more socially acceptable. Similar to FTLD types, they may also appear apathetic or uninterested in former hobbies or tasks.   Unlike AD, FTLD patients often develop motor problems early in their illness, particularly the FTD/ALS type.

It’s complicated….

The GOOD News

Ten percent of those with a familial gene for FTD never experience symptoms. This is my hope.

To learn more about FTLD and its subtypes, I recommend this website.

Image Copyright: Andreus / 123RF Stock Photo

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