Diagnosis of disorder remains problematic
Patients do not show all symptoms, thus complicating analysis
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FRONTOTEMPORAL DEMENTIA is a complex disease that's just beginning to enter public consciousness the way Alzheimer's disease did about 20 years ago. Because it can begin with a language disorder, motor-control problems, inappropriate behavior or a series of bad decisions, it can be misdiagnosed as a psychiatric disorder, attributed to stress or simply ignored for a long time.
Families, close friends or co-workers may notice bizarre, even dangerous behavior in someone close to them, but ascertaining the cause or even the fact that the person is ill can take months -- and sometimes years.
Early symptoms may include speaking or comprehension difficulties to the point where interaction with others becomes limited, irritability, impairments in financial decision-making, inability to organize or solve problems, lack of sympathy, decline in cognition or lack of attention to appearance. Complicating the analysis is the fact that no single patient exhibits all of the symptoms.
To date, the only way to obtain a definitive diagnosis is during autopsy.
Consequently, detecting FTD remains more art than science. Magnetic Resonance Imaging and CT scans can help rule out a brain tumor and show cerebral atrophy in the frontal and temporal lobes, but these and PET scans "are not a means for making a diagnosis," said Tiffany Chow, an associate professor of neurology and geriatric psychiatry at the University of Toronto.
In other words, the brain can still look fairly good when patient behavior indicates otherwise. Neuropsychological tests can help, but Chow said that nothing is more effective than a complete examination and history of speech impairments and/or behavioral changes.
But this can also be problematic. Most clinicians agree that information from a patient is unreliable, said Geri Hall, a clinical professor at the University of Iowa College of Nursing, and an advance-practice nurse who has worked with hundreds of FTD patients since 1978. They may admit to limitations related to language, but as the disease progresses, lack of insight prohibits them from recognizing, or caring about, their own deficiencies.
Compounding the problem is the fact that primary-care physicians and neurologists in private practice may have limited knowledge of the disease and difficulty obtaining information. Family members can be reluctant to tell the truth, out of embarrassment or fear of repercussions from a now-volatile loved one. Those who haven't lived with the patient on a daily basis have little to report.
INFORMATION about the cause of FTD is limited, though the latest research indicates that a progranulin gene mutation on the 17th chromosome plays a role. Another important factor, the tau protein, also is prominent in Alzheimer's disease. Essentially, misfolding proteins become like tangled train tracks that cause horrific wrecks in the brain. About 40 percent of cases are believed to have a genetic component. The rest are random.
Because there is no treatment or cure, clinicians address only the symptoms, often with medication to control behavior. Patients die from complications of the illness within three to 15 years.
To raise awareness, the University of California, San Francisco, Memory & Aging Center is working on a program they hope will air on public television in the next couple of years. In the trailer, a woman talked about her 49-year-old husband who took their young daughter into a rapidly flowing river, keeping her secure as she held the end of a stick under his control. Then he let go. For the safety of her children, she made the painful decision to put him in a nursing home.
COURTESY KAREN SHUSTER
Neurologist Bruce Miller points out a painting by Honolulu artist Peggy Chun, who has Lou Gehrig's disease, at the fifth annual Conference on Frontotemporal Dementias in San Francisco earlier this month. Miller says the disease has strong ties to FTD.
Typically, this leads to a new set of problems. Because patients are often young and physically able, they are too strong for staff members prepared only for the frail elderly -- especially if the FTD patient becomes moody and aggressive. When asked if her patients are expelled from nursing homes, Jill Shapira, a nurse practitioner who has worked with hundreds of FTD patients at UCLA, said simply, "Big time."
If there is any good news, it's the research that neurologist Bruce Miller, director of UCSF's Memory & Aging Center, has conducted on the artistic talents elicited in some patients during the course of this illness. At the International Conference on Frontotemporal Dementias in San Francisco earlier this month, Miller led a tour of patient work in the Yerba Buena Center for the Arts, where paintings by Hawaii artist Peggy Chun were featured. Chun has ALS (Amyotrophic Lateral Sclerosis, or Lou Gehrig's disease), an illness with "strong ties" to FTD, according to Miller.
"The dark ages is where we've been until recently," Miller said. "My hope is that within the next year, we'll see the beginnings of effective treatment for FTD."
In the meantime, families struggle to understand an illness with multiple components and an assortment of severe and subtle symptoms that essentially do one thing: destroy the essence of the person they love. "He's here, but he's not," a woman in the UCSF video explained. "It's a continual, day-to-day grieving process."
TYPES OF FTD
Frontotemporal Dementia (also called Frontotemporal Lobar Degeneration) is an umbrella term that covers several disease subtypes. They are named according to the way the disease began. All doctors agree that as each one progresses, though the severity of symptoms may vary, they all begin to resemble one another.
» Primary Progressive Aphasia or Progressive Non-Fluent Aphasia: Begins with language disorder and may remain isolated there for up to two years. All patients suffer behavioral symptoms and cognitive decline as the disease spreads.
» Semantic dementia: Language problems initially, primarily with comprehension.
» Pick's Disease: Immortalized in a tragic plot turn in Sue Monk Kidd's "The Mermaid's Chair" and first described by Arnold Pick in 1892. Behavioral symptoms exhibited first.
» Corticobasal Degeneration: Begins with motor control and coordination problems.
» Progressive Supranuclear Palsy: A related disorder that begins with problems with gait and balance.
» Association for Frontotemporal Dementias: www.ftd-picks.org; (866) 507-7222
» University of California-San Francisco Memory and Aging Center: www.memory.ucsf.edu
» UCLA: www.neurology.ucla.edu/FTDClinic.htm
» Northwestern University: www.brain.northwestern.edu
» Alzheimer's Disease and Related Disorders Association, Hawaii Chapter: www.alzhi.org; 591-2771
» Well Spouse Association: www.wellspouse.org; (800) 838-0879