When the family of show business legend Bruce Willis announced that he had been diagnosed with frontotemporal dementia (FTD), people scurried to the Internet to learn more about this fatal disease.
According to the Association for Frontotemporal Dementia (AFTD), it is the most common form of dementia for people under age 60.
Dr. Samina Kazmi, a board-certified neurologist who practices in Cleveland Clinic’s Tradition Hospital complex and is not involved in Willis’ medical care, says the average age of onset is 45-to-65, although the disease can present at a younger age.
“Between 10 percent and 20 percent of cases are genetically transferred,” says Dr. Kazmi. If one parent has this type of FTD, there is a 50-percent chance it will be inherited by their child.
Men and women develop FTD in similar percentages.
“Because it starts in the front of the brain – where behavior and language functions are controlled – some early symptoms include blunting of emotions, inappropriate behavior and trouble expressing yourself. The person affected may experience increasing difficulty in planning or organizing activities.”
The progression of symptoms varies by individual according to AFTD, but there is an inevitable decline in functioning.
“The length of progression varies from two to over 20 years,” Dr. Kazmi says.
Over time, FTD predisposes an individual to physical complications such as pneumonia, infection or injury from a fall. Average life expectancy is 7-to-13 years after the start of symptoms. The most common cause of death is pneumonia.
“Memory isn’t affected as first,” Dr. Kazmi says., “But no matter what part of the brain your neurological problem appears in initially, as it progresses, the entire brain will be involved.”
And although presently there is no cure, she adds, “anti-depressants and medications that can treat certain behavioral symptoms and improve quality of life are available.”
According to the National Institute of Health’s National Institute on Aging, there are several ways a doctor can diagnose frontotemporal dementia:
Perform an exam and ask about symptoms.
Look at personal and family medical history.
Use laboratory tests to help rule out other conditions.
Order genetic testing.
Conduct tests to assess memory, thinking, language skills, and physical functioning.
Order imaging of the brain.
A psychiatric evaluation can help determine if depression or another mental health problem is causing or contributing to the condition. However, only genetic tests in familial cases or a brain autopsy after a person dies can confirm with certainty a diagnosis of FTD.
Researchers are studying ways to diagnose FTD earlier and more accurately and to distinguish it from other types of dementia. One area of research involves biomarkers, such as proteins or other substances in the blood or cerebrospinal fluid which can be used to measure disease progression or the effects of treatment. Researchers are also exploring ways to improve brain imaging and neuropsychological testing.
Anyone concerned about their risk for FTD has the option of meeting with a genetic counselor, either individually or accompanied by a family member or friend. In some cases, the genetic counselor may recommend genetic testing to help clarify risk, but genetic testing is never a required part of genetic counseling.
A genetic counselor can evaluate the likelihood that a specific case has a genetic cause and help an individual think through the benefits and risks of genetic testing, ways to talk to family members about genetic testing, privacy concerns, and more.
Last spring, Willis was diagnosed with Aphasia, a disorder that affects how you communicate, hampering speech, writing, and understanding of both spoken and written language. But Aphasia is really just a term given to a set of symptoms.
It wasn’t until last month that the more specific underlying diagnosis of frontotemporal dementia was announced. After Willis’ condition was made public by his family, his wife, ex-wife and five daughters issued a statement which says, in part, “Bruce always believed in using his voice in the world to help others, and to raise awareness about important issues, both publicly and privately. We know in our hearts that – if he could today – he would want to respond by bringing global attention and connectedness with those who are also dealing with this debilitating disease and how it impacts so many individuals and their families.”
Dr. Samina Kazmi, a board-certified neurologist, is on the staff of Cleveland Clinic Martin Health. She earned her medical degree at Sindh Medical College, completed the residency program in the Department of Neurology at University of Mississippi Medical Center, and completed the fellowship program in Clinical Neurophysiology at West Virginia University. She is a Diplomat of the American Board of Psychiatry and Neurology, of the American Board of Clinical Neurophysiology and of the American Board of Vascular Neurology. She received her Florida medical license in 2010. She is accepting new patients at Tradition HealthPark One, 10050 SW Innovation Way, Port St. Lucie. Call 772-344-3811 or visit martinhealth.org/neurology for an appointment.