FTD is considered a pre-senile dementia, typically becoming symptomatic when patients are in their 50s and 60s. Its progression is thought to be slow; in fact, some experts suspect that the process may begin decades before symptoms become apparent.

Dementias are classified as frontotemporal when there is degeneration in one or both frontal or temporal lobes. Since FTD can affect one or both sides of the brain, and the brain's left and right hemispheres perform different functions, this leaves room for a significant variation in FTD symptoms. Experts now believe there are three distinct subtypes based on where the disease originates:

• Primary progressive aphasia begins in the brain's left hemispheres affecting language functions. Tiffany Chow, M.D., director of the Frontotemporal Dementia Clinic at the UCLA Alzheimer's Disease Research Center, explains that at least two types of primary progressive aphasia are known. When only the left frontal lobe is involved, the capacity to speak is affected. "Patients will slowly lose the ability to speak, even though they can still understand what they're hearing, which can make them terribly depressed and anxious," Chow says. On the other hand, when it's the left temporal lobe that's affected, fluency is maintained but patients begin to speak gibberish, and have difficulty understanding what others are saying - an equally frustrating experience. "Whether it's the temporal or frontal lobe type, primary progressive aphasia patients will at first say they're having a hard time finding the right word in conversation," Chow explains. "That happens to all of us occasionally, but in these patients it gradually becomes worse."
  
• While the left-sided FTD patients first display cognitive changes, right temporal variants tend to experience earlier and more pronounced alterations in personality and emotional processing - changes that can be mistaken for psychoses. "It's often misdiagnosed as mania or schizophrenia," says Paula Mychack, Ph.D., a researcher with the Alzheimer's Disease Research Center at the University of California, San Francisco. "Out of the blue these normal, law-abiding people begin exhibiting socially unacceptable behaviors, including criminal behavior, aggression and a profound lack of empathy toward their spouse. It's a terrible experience for family members because they don't understand what's going on. They think it's intentional, due to a mid-life crisis or something else." 
  Mychack and colleagues have been conducting the first studies using existing personality measures to systematically examine the changes in right-sided frontotemporal dementia patients. Among other things, the right-sided variants typically engage in compulsive behaviors, including constant eating and pacing, at least until later in the disease, when they become more docile.
  
• Finally, the third type of FTD affects both frontal lobes, producing a mixture of the right-sided and left-sided symptoms. It occurs with and without Pick bodies; and only the FTD patients with these bodies are said to have Pick's disease. "The Pick's patients often have problems with word finding," says Chow, "but they’re also having a hard time organizing and planning, and they start to show some disinhibited behaviors, all of which is happening gradually at the same time."

Difficult to Diagnose and Treat

When her husband first began acting strangely after losing his job, Joan Dunaway assumed he was depressed. She enrolled him in a depression study that included treatment with medication. But after six months, nothing had changed. She took him to see a doctor, who suggested that a mid-life crisis was to blame. On that physician's recommendation, the Dunaways sought marriage counseling. Still, nothing changed. So, Dunaway took her husband to another physician, who directed her to a neurologist. After a series of imaging tests, the neurologist arrived at the proper diagnosis: frontotemporal dementia.

Unfortunately, because so little has been known about FTD, and because its symptoms are so easy to confuse with something else - often Alzheimer's disease in the case of the primary progressive aphasia patients, or psychiatric problems in the case of right temporal variants - the Dunaways' experience was not unusual.

While the early signs of FTD are easy to mistake, physicians who have had experience with the disease know to look for features such as dramatic personality changes accompanied by progressive apathy and antisocial behaviors or language dysfunction, according to Bruce Miller, M.D., director of the Alzheimer's Disease Research Center at the University of California, San Francisco. Once suspicions are raised, Miller notes, neuropsychological exams and brain imaging scans usually capture the degeneration, facilitating an accurate diagnosis.

There are currently no treatments that are thought to reverse or even slow the process of frontotemporal dementia. The cholinergic drugs that delay the progression of Alzheimer's disease for some patients are much less likely to help FTD patients, who don't have a cholinergic deficit, Miller notes; indeed, he believes these medications can exacerbate FTD symptoms. On the other hand, Miller points out that serotonin- boosting antidepressants may actually be more effective in treating the symptoms of frontotemporal dementia patients than for Alzheimer's patients. It is the serotonin deficit that may result in the depression, anxiety and obsessive-compulsive behaviors of many individuals with FTD. In addition, antipsychotic medications may alleviate symptoms in FTD patients who are experiencing delusions or hallucinations.

"You have to target the symptoms," says Laura Mosqueda, M.D., medical director of the John Douglas French Center for Alzheimer's Disease. "Then, once you've determined whether the patient needs an antidepressant or an antipsychotic, you need to decide what side effects you want. For example, do we want an antidepressant that is somewhat sedating, or do we want to make the patient more active? Every drug has a side effect, but it may be used to the patient's advantage."

Mosqueda believes it's important to make family members aware that symptoms of depression, anxiety and psychosis may be alleviated through medication, and shouldn't merely be accepted as an inevitable consequence of the disease.

Still, medical treatments go only so far, and taking care of frontotemporal dementia patients presents a huge challenge.

Daily Caregiving Issues

One family caregiver, Dottie Price, remembers that in the early stages of her husband's illness, he would make purchases from telephone and door-to-door solicitors, and she had to spend quite a bit of time watching the checkbook and returning items he had bought. She would walk into the kitchen in the morning and find that her husband had taken food out of the refrigerator and left it on the counter during middle-of-the-night snack forays. She was forced to remove the knobs from the stove to prevent him from leaving the burners on.