From Odd Behavior to a Difficult Diagnosis

The tests of Joe Field's memory and cognitive ability yielded critical information: The frontal lobe of his brain clearly was not functioning properly. But no one knew why.

"I kept getting these vague answers like, 'Well, we can't really say what's wrong -- the only way we can be sure is on autopsy,' " Lynn Field recalled. "It was incredibly frustrating."

A second neurologist thought the problem might be undiagnosed bipolar disorder or substance abuse.

Lynn Field was skeptical. Her husband had been a social drinker and had never displayed psychiatric problems. She decided it was time to see a specialist, so she made an appointment with a neuropsychiatrist at Johns Hopkins.

After a 2 1/2 -hour appointment, the specialist delivered the grim diagnosis, one of an array of possibilities suggested by the Bethesda neurologist: Joe Field had frontotemporal dementia (FTD), a progressive and irreversible disorder also known as Pick's disease.

Although its symptoms are similar to early onset Alzheimer's disease, Pick's disease tends to shrink the frontal lobe of the brain. It is characterized as a rare disorder by the National Institutes of Health and affects about 200,000 Americans. Most cases are not inherited -- there is no history in Joe Field's family -- and the disease appears to be caused by an abnormal form of a protein that is deposited in brain cells.

Some victims are as young as 20, but the average age of onset is 54. Behavioral changes, cognitive decline and difficulty speaking are common; so is impulsive or inappropriate behavior.

Joe Field, for example, sometimes cries while watching the television show "CSI," his wife says; at other times he talks to himself. Neurologists say that some patients develop problematic behaviors such as blatant shoplifting.

Determined to do something beyond coping with the inevitable decline, the couple flew to California in December to participate in a research study at the University of California at San Francisco and to consult with experts.

"It's definitely a tough, tough disease," especially for families, said neurologist Bruce Miller, chief of UCSF's Memory and Aging Center, who evaluated Joe Field and met with the couple. Many cases, he said, are misdiagnosed for years as depression, severe anxiety, obsessive-compulsive disorder and late-life schizophrenia.

Like Joe Field, most victims seem relatively unbothered by the implications of their condition. "Generally it's not something I'm aware of," he said. "Most of the awareness that something is wrong is provided by people around me. That's good for me -- but not for the people around me."

Field is taking medications; the one that helps the most is Adderall, an attention-deficit-disorder drug. He still goes to work every day, although his responsibilities have been severely curtailed. A few weeks ago he took a short trip to the Canadian woods with a close friend.

Mostly he spends time with his wife and daughters. "They're afraid of the future, as am I," he said. "Lynn's been extremely supportive, but I know she struggles."

"This has been so hard," Lynn Field said. ·

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