Rare Brain Disorder Mimics Other Conditions

Rare Brain Disorder Mimics Other Conditions

(August 21, 2007) -- The initial symptoms were more annoying than alarming, Lynn Field recalls.

At age 47, Joe Field started having memory problems. After two years and many tests, he was finally diagnosed with Pick's disease, which shrinks the frontal lobe of the brain.

The Washington Post/Marvin Joseph

Her husband, Joe, had always been a bit forgetful, but during the summer of 2004 his memory got worse. Sometimes the 47-year-old electrical engineer said he didn't remember a conversation the couple had had the previous day. At other times he seemed distant; he stopped answering the phone in the afternoon, claiming that he was out of earshot or had turned off his cell phone.

Lynn, a Northern Virginia therapist, interpreted her husband's behavior as signs of midlife stress. Joe had been working long hours at the computer firm he founded with her brother. He had bouts of insomnia. Their daughters had left for college. At her behest, they started seeing a marriage counselor to help navigate what she believed was a rough patch in their 23-year marriage.

Looking back, Lynn says she wishes the problem had been so prosaic. She never imagined that the next two years of their lives would be consumed by consultations with nearly a dozen doctors on two coasts, scores of medical tests, days spent ferreting out information and arranging appointments, and sleepless nights worrying about the ominous possibilities. Doctors finally figured out what was wrong, but only after ruling out cancer, stroke, syphilis, narcolepsy, alcoholism, vitamin deficiency and a seizure disorder, among other things.

In November 2004, shortly after starting counseling and a few months after his annual physical, Field went to see Mark Plescia, his family doctor. He told Plescia about his insomnia, difficulty concentrating and general apathy.

"At the time it looked like textbook depression," recalled Plescia, who prescribed an antidepressant.

When that didn't help, Field consulted a psychiatrist, who switched his medication. The second antidepressant was equally ineffective.

Lynn was worried: Her normally serious husband had begun acting uncharacteristically "goofy." He often laughed at inappropriate things and put a denuded lamb chop bone in the refrigerator, then heatedly denied he'd eaten the chop.

When Joe returned to visit Plescia in April 2005, the doctor witnessed the troubling behavior. Joe kept repeating himself and seemed unable to focus. Plescia immediately ordered extensive blood work and sophisticated imaging of Joe's brain to check for a blockage, tumor or stroke.

The tests showed no abnormalities, so Plescia sent Field to a neurologist. She ordered brain-wave monitoring, a spinal tap and a sleep study. All results were normal, but Field was getting worse, although he often seemed fine to those who didn't know him. But his productivity had slipped dramatically at work and at home his behavior remained episodically odd.

Nearly a year after the first symptoms surfaced, Lynn decided Joe needed a neuropsychological workup, which could reveal problems that had eluded medical tests.

The tests of Joe's memory and cognitive ability showed 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 recalls. "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 2 1/2 -hours, the specialist delivered the grim diagnosis: Joe Field had frontotemporal dementia, 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, 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.

Determined to do something beyond coping with the inevitable decline, the Fields flew to California in December to participate in a research study at the University of California, 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. Many cases, he said, are misdiagnosed for years as depression, severe anxiety, obsessive-compulsive disorder and late-life schizophrenia.

Like Joe, 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."

Joe 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.

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."

By: Sandra G. Boodman
Source: The Washington Posts

Last updated: 08/07

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Rare Brain Disorder Mimics Other Conditions