A loved one turns 50 (or so),
impulsively buys a flashy sports car that he can’t
afford, aggressively propositions women in
restaurants, swears at his boss, then lobs insults
at you when you suggest that he settle down. You
might think that he’s having a somewhat tardy
midlife crisis...but he’s not.
The real problem may be a type
of dementia called frontotemporal degeneration (FTD)
that leaves memory intact while causing disturbing
changes in behavior, personality and/or language.
FTD occurs equally in men and women. People with
this progressive brain disorder often alienate
friends and family, jeopardize their jobs, maybe
even get into legal trouble—yet often they’re
completely unable to recognize that they have a
problem. And even if they do see their doctors,
they’re likely to be misdiagnosed with depression or
some other psychiatric problem, at least in the
That’s why they need all of us
to be aware of the warning signs...so patients can
get the help they need and appropriate plans can be
made for their future. Here’s what you should know
RECOGNIZING AN UNRECOGNIZED DISORDER
One reason why FTD often goes undiagnosed is that it
tends to strike earlier than other forms of
dementia, typically developing in a person’s 50s or
60s—which people may think of as “too young” for
dementia. Alzheimer’s disease, in comparison,
appears 10 to 13 years later, on average. Another
source of confusion is the fact that FTD patients
usually ace memory tests used to detect
Alzheimer’s—for instance, they often have no trouble
keeping track of day-to-day events and staying
oriented to space and time. Also, many people have
never heard of FTD (also called frontotemporal
dementia or Pick’s disease) even though it accounts
for up to 20 percent of all dementia cases.
The term FTD actually
encompasses several related disorders, all of which
are characterized by progressive damage to the
frontal lobe, a brain area associated with
decision-making and behavior control...and/or the
temporal lobe, which is associated with emotion and
Though genetics plays a role in
about 15 to 20 percent of FTD cases, the majority of
cases do not appear to be inherited. Doctors aren’t
sure what triggers FTD, though they do know that in
some cases the disorder occurs when a protein called
tau degenerates and is no longer able to perform its
main function, which is to stabilize the structure
of brain cells, said Bradford C. Dickerson, MD, an
associate professor of neurology at Harvard Medical
School and director of the Frontotemporal Disorders
Unit at Massachusetts General Hospital. Other cases
result from changes in other proteins, he added. As
a result, neurons die or become shrunken and
misshapen...scar tissue forms...and there is an
accumulation of abnormal protein within brain cells.
As a consequence of the brain
degeneration, patients experience progressive
changes in behavior, language and/or motor skills.
Symptoms may include any or all of the following...
changes—including loss of empathy...heightened
aggression...and increasingly inappropriate
social behavior (hypersexuality, excessive
swearing, laughing at others’ misfortunes,
distractibility, poor judgment and impaired
decision-making skills. Examples: Impulsive
spending or financial risk-taking...inability to
plan and prepare a meal...inattention to
personal hygiene...driving like a reckless
Compulsive behaviors, such
as repetitive hand clapping, incessant humming
or shoplifting...and intense cravings for sweets
or other carbohydrates.
Apathy, lack of motivation,
listlessness, irritability—symptoms easily
mistaken for depression.
Increasing problems with
language, such as difficulty naming familiar
objects, expressing thoughts and comprehending
words. Ultimately, some patients are rendered
incapable of speaking...and some lose the
ability to comprehend the speech of others.
Muscle rigidity, weakness
and tremors, which can lead to trouble balancing
and walking. Early on, such symptoms often are
misconstrued as signs of Parkinson’s disease.
Eventually, patients become unable to perform
Sadly, FTD is ultimately fatal,
with the duration of the disease ranging from two
years to more than 10 years.
It’s tragic to think that,
during the early stages, an undiagnosed patient
might so alienate his/her family and friends—who are
understandably bewildered and upset by the person’s
disturbing behavior—that he winds up alone during
the later stages, with no loved ones to help him.
That’s one reason why a diagnosis is so crucial.
“In many people with FTD, the changes in the brain
reduce insight and self-awareness to a degree that
the patient is not able to recognize his
impairments,” Dr. Dickerson said. So if you suspect
that a loved one has FTD, insist on accompanying him
to see his doctor and request a referral to a
neurologist with expertise in the disorder. No
single diagnostic test exists to confirm or rule out
FTD. Diagnosis is based on a detailed cognitive and
neurological exam...a neuropsychological exam to
assess behavior, language and decision-making
functions...and neuroimaging tests such as MRI or
PET scans to check for atrophy in various areas of
There is no known way to cure
FTD or stop the progression of the disease, but
there are treatments that can help ease some of the
symptoms. For instance...
serotonin reuptake inhibitors may be able to
help control aggressive behaviors, impulsivity
and carbohydrate cravings.
mood-stabilizing medications may be able to help
manage irrational or compulsive behaviors and
ease agitation. However, these drugs can have
serious side effects, including accelerating
heart disease, so the benefits need to be
carefully weighed against the risks, Dr.
Speech and language therapy
can help a patient learn alternative ways to
communicate (such as with gestures or by
pointing to pictures), reducing frustration and
improving quality of life.
Occupational therapy makes
it easier for a patient to perform basic
activities of daily living and to avoid falls.
Counseling or psychotherapy
rarely helps FTD patients, Dr. Dickerson
noted—but it almost always helps their families.
FTD eventually progresses to the
point that patients need 24-hour care. The sooner a
patient is properly diagnosed, the sooner plans can
be put in place to make his life as secure and
comfortable as possible.
For additional information,
visit The Association for Frontotemporal
Degeneration (theaftd.org). This organization can
also help you find doctors with expertise in
FTD...as well as programs that provide FTD
caregivers with invaluable support.