RAY SUAREZ: Now, diagnosing and treating dementia. NewsHour correspondent Spencer Michels reports for our Health Unit, a partnership with the Robert Wood Johnson Foundation.
SPENCER MICHELS, NewsHour correspondent: Keith Jordan, an environmental activist and software innovator — shown here several years ago — suffered from an often misdiagnosed neurological condition that attacks the front part of the brain and produces very strange behavior. Doctors say the condition is more common than previously thought.
Eventually Jordan’s brain atrophied so much that his body’s motor systems stopped working, and he died last May.
CASSANDRA SHAFER, wife of dementia patient: We loved to go to the farmer’s market. That was his favorite thing in the world.
SPENCER MICHELS: Both Jordan and his wife, Cassandra Shafer, who raised their family in Northern California, were slow to realize he was actually sick, a common problem with many dementias. She says his disease — finally identified as frontotemporal dementia, or Pick’s disease — affected the whole family.
CASSANDRA SHAFER: A lot of rage, a lot of guilt, a lot of confusion, a lot of sorrow.
SPENCER MICHELS: Before he died at age 52, Keith Jordan was videotaped as part of a research project looking into the bizarre and often crude behavior that is one hallmark of frontotemporal dementia or FTD.
KEITH JORDAN, Dementia Patient: I love to fart loud and long and clear. I love to fart. It’s getting worse every year.
SPENCER MICHELS: Dr. Bruce Miller, who was Jordan’s physician, directs the memory and aging center at the University of California at San Francisco.
DR. BRUCE MILLER, University of California, San Francisco: This is a disorder that attacks the front part of the brain, the part of the brain that is so important for social regulation. So often these people end up being called psychiatric, or misbehaving, or antisocial and never actually get in for a diagnostic evaluation.
SPENCER MICHELS: Jordan's wife remembered that, at first, her husband began moving his hand weirdly and telling jokes and making puns.
CASSANDRA SHAFER: Over and over the same joke, four times at dinner, and every time as if for the very first time. And when I'd talk with him, he'd respond in puns rather than legitimately to the conversation that we were having.
SPENCER MICHELS: But the symptoms were no joke. Jordan, who is seen here in an old home video, took his 6-year-old daughter swimming to the Russian River.
CASSANDRA SHAFER: And then he sort of pulled her into the river with the stick, you know, out into where the water was deep over her head, fast current, and then he dropped the stick, and swam away.
SPENCER MICHELS: Cassandra Shafer was nearby and rescued their daughter.
What would he say when you asked him about these events?
CASSANDRA SHAFER: Well, that's the part where the weirdness comes through, or the jerk part, whatever you want to say, is he'd just look at me blankly like, "Big deal."
SPENCER MICHELS: For two years, doctors, psychologists, therapists, and others couldn't figure out the symptoms. A series of drugs and therapies, even an exorcism, had no effect until finally Jordan was referred to UCSF.
Dr. Miller says that health professionals all too often miss a dementia diagnosis, especially the rarer forms.
DR. BRUCE MILLER: In U.S. medical schools, there are places that don't even teach about the frontotemporal dementias and have minimal teaching about Alzheimer's disease. These are the most costly diseases in our society, so that these really have to be emphasized.
Raising FTD's profile
SPENCER MICHELS: But Miller and other researchers now believe dementia is far more prevalent among Americans than the official estimate of 5 million.
DR. BRUCE MILLER: We think that this greatly underestimates the number of people who are in the early stages of these diseases. And it may be at least twice as many people have not yet been diagnosed with dementia, but are just beginning to show problems related to these diseases.
SPENCER MICHELS: New Mexico Sen. Pete Domenici was recently diagnosed with frontotemporal dementia, raising the disease's profile.
In his research, Miller discovered that patients with FTD often become amazingly creative, taking photographs, painting, making artificial flower arrangements.
For this 56-year-old Bay Area patient, who asked not to be named, the changes to her brain have led to a spree of poetry-writing combined with an intense spiritual appreciation of nature.
DEMENTIA PATIENT: The flow of nature lifts my heart.
SPENCER MICHELS: Did you always think so highly of nature, get involved in nature, or is that something new?
DEMENTIA PATIENT: It is something new. It is something new.
SPENCER MICHELS: And do you know what it's connected to, as to why that's happened at this point in your life?
DEMENTIA PATIENT: Well, it's connected that I am looking at nature in a deep way.
SPENCER MICHELS: Her husband says doctors say his wife has semantic dementia, a form of FTD. The patient doesn't think of herself as sick, just slow.
Do you feel healthy or do you feel anything is different than it used to be?
DEMENTIA PATIENT: That I can see nature purely and write these incredible poems, I'm so grateful for that, so that slows me down.
DR. BRUCE MILLER: You see this subtle, slow change in the person, their sense of self, their personality, their behavior, how they empathize with other people.
Researching dementia medication
SPENCER MICHELS: Researchers are looking for drugs that might have some effect. In this lab, they are testing thousands of substances on the chance that one might prove effective, but progress has been slow.
And that frustrates Dr. Stanley Prusiner, who was awarded the Nobel Prize in Medicine in 1997 for his discovery of a disease-causing brain protein called a prion.
DR. STANLEY PRUSINER, University of California, San Francisco: The last time we've made a really meaningful advance in the treatment of these chronic degenerative brain diseases was 1967.
SPENCER MICHELS: That was the introduction of L-DOPA for Parkinson's disease. The progress since then has not been on the drug front.
Prusiner is still searching for drugs to modify the proteins and fight these diseases. Researchers' hopes have been dashed several times, but they haven't given up.
DR. STANLEY PRUSINER: Do we have a medicine sitting in the next room that we're excited about? A year ago, I would have told you yes. That one went down the tubes. But now we're beginning to put together a true drug discovery program.
SPENCER MICHELS: When most people think of dementia, they often think of Alzheimer's disease, which in fact accounts for about half of degenerative brain diseases. Prusiner now believes that finding a key to any one of the rare brain diseases may lead to a breakthrough in Alzheimer's.
DR. STANLEY PRUSINER: It's become clear to me that, if we could cure one of these diseases, any one of them, it would provide a road map to the others.
SPENCER MICHELS: At UCSF, researchers are doing drug evaluations on Alzheimer's patients like 58-year-old Regina Karp. Although she wasn't diagnosed correctly for seven months, she is now part of a study to see if an experimental drug will block dangerous proteins that doctors suspect cause Alzheimer's.
Researchers will take pictures of her brain over time using a magnetic imaging machine. Dr. Adam Boxer is the principal investigator.
DR. ADAM BOXER, University of California, San Francisco: We know that a normal person with Alzheimer's disease, their brain may shrink a few percentage points over six months, and we hope that this drug, if she receives it, will stabilize that shrinkage. And we think the shrinkage occurs from damage to the actual neurons in the brain.
SPENCER MICHELS: Boxer is more optimistic than some of his colleagues that drugs for treating dementia, especially Alzheimer's, are not far off.
DR. ADAM BOXER: And so what we're seeing now is that treatments that are really aimed at the biology of the disease, the toxic proteins that we think kill brain cells in Alzheimer's disease, they've now made it out of the laboratory and they're starting to be tested in humans.
And I think we're getting some early signals that some of these approaches might actually work.
SPENCER MICHELS: Whether and when these approaches can cure or modify dementia are still open questions, but scientists are convinced that they can progress in that direction, only if they have enough early-onset patients to become part of the research.