A Very Special Brain

<B>Morley Safer</B> Reports On People Diagnosed With Williams Syndrome

The brain, the most complex of all organs, defines us in more ways than we can even think about. There are about 35,000 genes in the brain, and if only a few of those are missing, the result can be devastating and fascinating.

Williams Syndrome is a rare birth defect in which about 20 genes are missing. Six years ago, correspondent Morley Safer reported on people diagnosed with Williams Syndrome. Their stories were so fascinating that 60 Minutes II decided to go back to see how they're doing.

But first, here's a look back at 1997 in this 60 Minutes classic.
Gloria Lenhoff remembers more than 1,000 songs, yet she can't add 5 plus 4. Michael Williams can play almost anything, yet he can't go out the door without getting lost. Meghan Finn studies music in college but can't tell left from right.

60 Minutes met these remarkable people at a music camp in Massachusetts -- people with profound disabilities but with an equally profound passion for music. They often have unusual, almost elfin features. Their IQs average in the 60s, but they have the social skills of talk show hosts, what's been called "cocktail party personalities."

To a stranger, any sense of their handicap quickly disappears, and one is overwhelmed by their friendliness and openness.

"Music is a huge part of my life. To me, music is like soup. Music comes down to your throat. It feels so warm. So music is like soup. It tastes good," says Meghan.

Gloria says she can sing in 25 languages: "I could sing in Macedonian, Korean, Yiddish, you name it."

Among the things that seem to be common among people with Williams is extremely sensitive hearing. It's remarkable. They can even hear whispering.

But part of that really sensitive, acute hearing is a negative reaction to loud noises. For example, thunder sounds like a cannon going off to Meghan: "Just shot off, right next to our house, and it made me cry so hard."

For the most part, they are among the happiest people you've probably ever met. And they can't stop smiling. But are they really happy most of the time?

"Yes, I am," says Bob. "I am, too," adds Jason Dennis.
Michael Williams had always known he was different, but he only learned last year that his condition had a name. For the first time, at the music camp, he met other people just like him.

"Somehow, I felt that I fit in," says Michael, who is most comfortable at the piano. But as a child, his parents never dreamed that he would be capable of handling the complexities of the instrument.

"My other kids had piano lessons. He didn't, we didn't think he could do it. After they went out to play, he would sit down – for hours on end, for days on end. And all of a sudden, one day he played a song," says Frank Williams, who admits he was devastated when his son was born and he clearly wasn't normal.

But that changed a long time ago. "We were blessed," says Michael's father.

"The opportunity to study an individual with Williams Syndrome is exciting. It provides a window on brain development," says Dr. Barbara Pober, who runs a Williams Syndrome clinic at the Yale University School of Medicine. "We can learn an awful lot about what it means to have a handicap from folks with Williams Syndrome."

And what it means to be shunned and isolated. Last year, Meghan tried living in a college dorm. While she did make a few friends, her mother, Liz Costello, says she had a rough time: "She doesn't fit in. She knows she doesn't fit in. That's very hard."
Since that story first aired, we've learned more about Williams Syndrome. Most people who have it are not only using different parts of their brain when they listen to music, they're using more of it than the rest of us. And they are more engaged emotionally.

Michael Williams is still most comfortable at the piano. 60 Minutes II caught up with him in September performing at a retirement home in upstate New York.

We also went back to see some of our Williams friends when they got together for one last time at that music camp in Massachusetts. Ben, a very grown-up 17-year-old, plays the drums. Gloria has made two CDs, and now sings in 30 languages. Jason is playing the drums and working part-time at a video store.

And Meghan is living in California, and attends regular classes at a local college, where she's learned to ignore people who make fun of her. "I'm not gonna be pushed or shoved or anything like that – because nothing's gonna pass me by. I'm a tough woman," she says.

And, we met two new faces, both pianists and composers: Tori, 22, and Alec, 18. Alec was barely two months old when his mother, Lori Swaezey, sat him down next to her while she played a jazz piece on the piano.

"When I came to a certain whole note in the piece, he matched the note, and just sang, 'Ooh.' I looked at him and he smiled like crazy and was kicking his legs," recalls Swaezey. "I couldn't believe it. I knew there was some very spooky connection with music at that point."
While there's no question that those 20 missing genes can have a devastating effect on the lives of people with Williams, it also seems to create an undeniable bond among them.

"When I'm with, like, all of these people that are around me right now, I feel like I fit in," says Tori. "I feel safe. All these guys have taught me not be negative and you know, I'm just happy."

And they have remained positive through tremendous adversity. In Ben's short life, he's already been through five major surgeries to correct heart and spinal problems – problems that can be common for people with Williams. His mother, Terry Monkaba, told Safer that music got him through a very tough, young life.

"From when he was very small, music was the thing that calmed him down. And music is what taught him to walk. He didn't walk, he marched to music when he was 4-and-a-half," says Monkaba, who admits it's been difficult for them as a family. "Benjamin has a two-years younger brother who has really been angry for many years because he was cheated out of a regular brother. So it makes family life, you know, very difficult."

When we last met Dr. Pober, she was running a Williams Syndrome clinic at Yale. In June, she left for further genetic study, but her connection with Williams patients remains strong. Since our first story, the human genome project has been completed. Geneticists now know that most people with Williams are missing the same 20 genes. And they think they know why they are so engaged by music.

"There was a very recent study that had individuals with Williams Syndrome listen to music, and then had controlled individuals listen to music and compared the areas of the brain that were activated," says Pober. "Folks with Williams Syndrome activated more brain areas and not the same area that you or I would activate. So, it was an overall greater activation … The underlying differences could explain why persons with Williams Syndrome are so much more connected to music. Seem to respond to music differently."
The great tragedy of Williams, however, is that they know they're ill. "They're smart enough to know they're different. And that is a great hardship for most adolescents and adults," says Pober.

In Meghan's case, her mother says she's accepted that she will never have children, but still has great anxiety about being alone.

"She said to me a couple of years ago, 'You know, mom, I just wanna have someone that I can grow old with, too. And have somebody, you know,'" says Liz Costello. "And so, she has a friend who has Williams Syndrome who's, she's engaged to be married. And she said, 'Part of me is so happy and the other part of me is so sad 'cause I don't have anyone.' But you know, who knows what the future's gonna bring? You know, who knows?"

Last summer, with her mother looking on, Meghan recorded a song for her own CD. "I sometimes ask myself why are we going through this CD thing, but boy, it's magical. It's just magical," says Meghan's mother.

"She has a disability. She's not a savant. There are many things she can't do, she won't be able to do. But you know, she has something that when she sings, people love to listen to her."


  • Rebecca Leung

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