On the left are MRI images of the normal human brain and on the right are images of a human brain affected by Frontotemporal Dementia (FTD), which shows brain atrophy (shrinking). As FTD progresses, it kills off cells effectively causing the brain to shrink. (Photo courtesy of sageke.sciencemag.org)
On the left are MRI images of the normal human brain and on the right are images of a human brain affected by Frontotemporal Dementia (FTD), which shows brain atrophy (shrinking). As FTD progresses, it kills off cells effectively causing the brain to shrink. (Photo courtesy of sageke.sciencemag.org)
DELPHOS — Frontotemporal dementia (FTD) is a form of irreversible dementia which affects 50,000-60,000 nationwide, accounts for 2-5 percent of all dementia cases and affects more men than women.

The Association for Frontotemporal Degeneration’s (AFTD) Executive Director Susan Dickinson said FTD is a neuro-degenerative brain disease that does not affect the memory as Alzheimer’s does; instead, it affects parts of the brain that controls behavior, language and movement.

“People with FTD are affected at a much younger age,” she explained. “The average age is 57, 10 years younger than an Alzheimer’s diagnosis.”

She said people diagnosed with FTD at this age are still working and parenting kids and teens at home. The onset of the disease often occurs in people between the ages of 50 and 60, but has been seen as early as age 21 and as late as age 80. Roughly 60 percent of cases occur in people 45-64 years old.

“It’s an uncommon disease that not many people understand,” Dickinson detailed. “Many physicians are unaware of the disease which results in misdiagnoses or delayed diagnosis.”

She said some FTD patients are misdiagnosed with psychiatric disorders, including Alzheimer’s and depression. No diagnosis or a delay in diagnosis occurs frequently since the symptoms presented are not memory-based.

“It’s that many more years the patient and their family has less together than if there would have been a diagnosis on the onset,” she said. “It affects their personalities and behaviors which can do a lot of harm to relationships.”

FTD is caused by progressive cell degeneration due to protein-filled structures (Pick bodies) that clump together and become toxic to brain cells causing them to die in the frontal lobes (the areas behind the forehead) and/or temporal lobes (the regions behind the ears). Brain cell death leads to tissue shrinkage and reduced function in both lobes which control planning and judgment, emotions, speaking and understanding speech and certain types of movement.

“The temporal lobes of the brain control language and the frontal lobes affect personality,” Dickinson clarified.

Research from the University of California in San Francisco revealed 60 percent of people develop FTD with no known cause and the other 40 percent have a family history of the condition.

According to AFTD, the majority of FTD cases are not being inherited within the family; however, people with both inherited and sporadic (not inherited) FTD exhibit the same clinical symptoms, which makes evaluation of the family history the most sensitive tool for determining the likelihood of a genetic cause.

“In 15-20 percent of the cases, there is clearly a hereditary link, a vertical pattern including a grandparent, parent and their offspring. Familial cases where there is no clear hereditary pattern — but includes at least one other relative who has or had a neurodegenerative disease — occur 30-35 percent of the time. Perhaps there’s a smattering of Parkinson’s or another inheritable disease that elevates the risk of FTD,” Dickinson said. “The other 50 percent of FTD cases are sporadic (not hereditary) and there is no understood cause.”

Alzheimer’s.org reports FTDs can be grouped into three types — as defined by the earliest symptoms physicians identify when they examine patients — and includes personality/behavior decline, progressive language decline and progressive motor decline.

Personality/Behavior decline behavioral variant FTD (bvFTD) is characterized by changes in personality, behavior, and judgement — which can result in embarrassing social situations — and a numbing of emotions.

“It isn’t that the patient does not love their family any longer, they just can’t show it,” Dickinson said. “This disease can rip families apart.”

The progressive language decline Primary Progressive Aphasia (PPA) is marked by early changes in language ability, including speaking, understanding, reading and writing. There are three identified variants.

• Semantic PPA (semantic dementia) causes a person to slowly lose the ability to understand single words and sometimes the ability to recognize the faces of familiar people and common objects;

• Agrammatic PPA (progressive nonfluent aphasia) results in a person having more and more trouble producing speech; and

• Logopenic PPA symptoms includes a person having trouble finding the right words during conversation but they can understand words and sentences. The person does not have problems with grammar.

Progressive motor decline Corticobasal syndrome (CBS), progressive supranuclear palsy (PSP) and amyotrophis lateral sclerosis (ALS) are characterized by difficulties with physical movement, including the use of one or more limbs, shaking, difficulty walking, frequent falls and poor coordination.

• CBS can be caused by a gradual atrophy and loss of nerve cells in specific parts of the brain causing progressive loss of the ability to control movement, typically beginning around age 60. The most prominent symptom may be the inability to use the hands or arms to perform a movement despite normal strength (called apraxia);

• PSP inflicts problems with balance and walking and causes people to move slowly, experience unexplained falls, lose facial expression, and have body stiffness, especially in the neck and upper body — symptoms similar to those of Parkinson’s disease. A hallmark sign of PSP is trouble with eye movements, particularly looking down; and

• FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig’s disease. Symptoms include the behavioral and/or language changes seen in bvFTD as well as the progressive muscle weakness seen in ALS. Symptoms of either disease may appear first, with other symptoms developing over time.

“People with FTD typically live 7-8 years and the wide ranging prognosis of 2-20 years is the best clinical experts can give,” Dickinson said. “ There is one exception, the prognosis for a patient with FTD with an ALS overlap is currently 2 years.”

Key differences between FTD and Alzheimer’s includes:

• Most people with FTD are diagnosed in their 50s and early 60s and only about 10 percent are diagnosed after age 70. Alzheimer’s grows more common with increasing age;

• Memory loss tends to be a more prominent symptom in early Alzheimer’s than in early FTD, although advanced FTD often causes memory loss in addition to its more characteristic effects on behavior and language;

• Behavior changes are often the first noticeable symptoms in bvFTD, the most common form of FTD. Behavior changes are also common as Alzheimer’s progresses, but they tend to occur later in the disease;

• Problems with spatial orientation — for example, getting lost in familiar places — are more common in Alzheimer’s than in FTD;

• Problems with speech. Although people with Alzheimer’s may have trouble thinking of the right word or remembering names, they tend to have less difficulty making sense when they speak, understanding the speech of others or reading than those with FTD; and

• Hallucinations and delusions are common as Alzheimer’s progresses, but relatively uncommon in FTD.

FTD itself is not life-threatening. It does, however, predispose patients to serious complications such as pneumonia, infection, or injury from a fall. Most patients die from problems related to advanced disease and the most common cause of death is pneumonia.

For more information visit www.theaftd.org, www.facebook.com/TheAFTD, or twitter.com/AFTDCure. To view a film documentary highlighting four families living with FTD, go to youtube.com/watch?v=drgzhKe_YWI.