Transcript: A beautiful mystery -- 60 Minutes: Australia

August 8, 2004

Reporter: Peter Overton
Producer: Lincoln Howes

INTRO
PETER OVERTON: They're the happiest, friendliest, most lovable people you're likely to meet. Just being around them is a joy, an absolute delight. But there's something slightly wrong. They suffer from a rare and baffling genetic disorder called Williams Syndrome, what the scientists call a beautiful mystery. The Willies, as they're affectionately known, also have low IQs, but they can still teach the rest of us a thing or two. They could just hold the secret to that age-old argument of nature versus nurture, the secret of what makes us tick, what makes us the way we are.

STORY
PETER OVERTON

PAUL NOTMAN: I'm outgoing, I'm funny and I love dancing.

PETER OVERTON: Paul Notman is 16. He likes football, cricket and dance music. He's the most popular kid in his country high school, yet Paul is not like his mates.

You strike me as a very happy person.

PAUL NOTMAN: Yes, I am.

PETER OVERTON: Are you always happy?

PAUL NOTMAN: Yes.

PETER OVERTON: Sarah Noone can't add five and three, but she knows every word that Lennon and McCartney ever wrote. These are the special mysteries of a special condition. It's called Williams Syndrome.

GERALDINE NOTMAN, MOTHER: They're very friendly, almost to the extreme. There's no prejudice, there's no religion or anything that comes into meeting people. They don't care who they are, whether they're rich or poor, fat or skinny, short or tall, black or white.

PETER OVERTON: Williams Syndrome sufferers are missing 26 genes, they have low IQs and distinctive elfin features. But since the syndrome was identified 40 years ago, other traits have become evident. The Willies, as they're known, are sensitive to noise and love music. And they're extremely sociable, blessed with friendly, outgoing natures. In fact, they have what's referred to as cocktail party personalities.

BILL NOTMAN, FATHER: He was the sort of baby that probably only a mother could love him. He wasn't the best looking fella as a little bloke, big mouth and huge eyes which he actually grew into, didn't he?

PETER OVERTON: Like many people, Paul Notman's parents, Bill and Geraldine, had never heard of Williams Syndrome when he was diagnosed at four-and-a-half.

GERALDINE NOTMAN: You think, you know, why did it happen? Why us? It's the hows, the whys, is there someone to blame? Is it either of us? Is it something we've done?

PETER OVERTON: In fact, the genetic disorder that causes Williams Syndrome is completely random. It's called a beautiful mystery. Beautiful, because of the irrepressible personalities it creates. By the time Paul hit kindergarten, he was already the life of the party.

PETER OVERTON: Do you like to be surrounded by people?

PAUL NOTMAN: Yeah.

PETER OVERTON: Why's that?

PAUL NOTMAN: I don't know, just ... makes me excited, because I have so many friends and they care about me and I appreciate that, too.

PETER OVERTON: Do they love you?

PAUL NOTMAN: Yeah. Everyone does.

PETER OVERTON: Sarah, too, displays the sociable, outgoing nature that makes Williams Syndrome children a joy to be around.

ROSEMARY NOONE, MOTHER: They love other people being happy, relate probably better to adults than other children, because there's more of an understanding there. But there's a real empathy for people who are sad or upset.

SARAH NOONE: This is my mum and she's cuddly, nice, and I love her very much.

PETER OVERTON: This beautiful mystery affects just one in 20,000 people and it's often associated with physical defects like heart problems, but it's through their outgoing personalities that people like Sarah and Paul may yet be able to teach us something about ourselves. Indeed, they may hold the key to what makes us the way we are.

MARILEE MARTENS, RESEARCH SCIENTIST: So if you see a picture of someone that you think looks very friendly and you for sure would like to go talk with them, then you would answer this one.

PAUL NOTMAN: Definitely would.

MARILEE MARTENS: That's right.

PETER OVERTON: Marilee Martens of Melbourne University has just completed the most comprehensive study of Williams Syndrome ever undertaken.

MARILEE MARTENS: Would you like to go up and talk to that person?

PAUL NOTMAN: Definitely would.

MARILEE MARTENS: Would you like to talk with him?

PAUL NOTMAN: Definitely would not.

PETER OVERTON: She set out to discover what makes people like Paul so friendly.

PAUL NOTMAN: Yes.

MARILEE MARTENS: Definitely?

PAUL NOTMAN: Definitely would. She's got a big smile and she looks friendly.

MARILEE MARTENS: Peter, this is a slice of the human brain and the structure that's coloured there in blue is the amygdale, which is believed to be one of the critical structures that's associated with what we would term sociability or friendliness. So we're looking at that structure in individuals who have Williams Syndrome to see if we can help make a link between brain structure and how that affects behaviour.

PETER OVERTON: Paul and 28 other Williams Syndrome individuals underwent brain scans, the largest test group ever assembled in the world. What the test revealed was the amygdale, the part of the brain which dictates our sociability, was larger in the Williams Syndrome subjects. The discovery may unlock the age-old secret of whether our personalities are formed by nature or nurture.

MARILEE MARTENS: I would say they help give us a glimpse, into that. We do feel like our study is very significant in that we've been the first group that have actually measured out the volume, the actual size of the amygdale, in individuals with Williams and compared it to others. So it's just giving us a better picture of the relationship between the brain and behaviour.

PETER OVERTON: But as some secrets of Williams Syndrome are being revealed, others remain a tantalising mystery, for instance, the incredible affinity with music, another remarkable symptom.

ROSEMARY NOONE: As soon as a song comes on the radio, it doesn't matter what era it's from, Sarah's straight onto the words and has this amazing memory for music. It gives her the ability to go into her own world, which sometimes is a good thing. It's a release from all the pressures and difficulties that someone with a disability has trying to keep up with everyone else. In her own world, she can control it and enjoy it and dance along and keep fit.

PETER OVERTON: In the United States, the Williams Syndrome affinity with music has been well documented. Gloria Lenhoff is the Williams Syndrome poster girl. She can sing in perfect pitch in 30 languages.

GLORIA LENHOFF, SINGER: Macedonian, Korean, Bulgarian, Yiddish, you name it.

ROSEMARY NOONE: What seems to be, for a lot of the children, that it's just innate and they have extra-sensitive hearing, hyperacusis, which means that they hear a lot more of the background of the music, they hear the harmony very strongly, they hear all sorts of other noises that we're able to block out. Sarah will often sing a harmony rather than a melody.

PETER OVERTON: That's harder, isn't it?

ROSEMARY NOONE: Yeah … well, for us, for most people it is, yes, but not for Sarah, that's what she tunes into often.

PETER OVERTON: Why do you love the Beatles so much?

SARAH NOONE: Oh, I love their rhythm.

PETER OVERTON: Their rhythm. And what else?

SARAH NOONE: Their voice.

PETER OVERTON: You're nearly as good as them.

SARAH NOONE: Oh, my god.

PETER OVERTON: One of the most poignant aspects of Williams Syndrome is that those who have it are aware of their limitations. They're astute enough to know that they're different.

PETER OVERTON: Does she ever or has she ever looked at you in the eyes and said, "Why am I like this?"

ROSEMARY NOONE: Yes. Yes, so there is that understanding.

PETER OVERTON: I guess the sadness about Williams Syndrome is that she's actually aware that she's different.

ROSEMARY NOONE: Yes. And I suppose like all parents, you know, I have my frustrations too, when it's difficult for Sarah to learn something or do something and Sarah often apologises to me for that.

PETER OVERTON: But that's probably more the nature of having Williams Syndrome, isn't it?

ROSEMARY NOONE: Yes. I think the children I've met, other children with Williams Syndrome, are pretty much the same. They feel sorry that they can't do more.

PETER OVERTON: Can you understand that you're not normal?

PAUL NOTMAN: Yes.

PETER OVERTON: So how does it make you feel knowing your friends are normal and you're not?

PAUL NOTMAN: I don't mind it, because everyone likes me and I'm just me.

YOUNG WOMAN: It's hard for people to understand what we are going through and how sometimes we are happy but sometimes we aren't because we go through hard stages in our life.

TEENAGE GIRL: They discriminate and they do things that are wrong towards us and I would personally like it to not happen any more.

PETER OVERTON: What sort of things do they do that are wrong?

TEENAGE GIRL: They look at you, they stare at you, they just do things that are uncomfortable.

TEENAGE BOY: So difficult being teenager for me, because I get hard times at school, hard times at home. Don't pick on me, I don't like it at all.

PETER OVERTON: It's only been four decades since Williams Syndrome was identified and much is still a mystery. Even life expectancy is unknown.

ROSEMARY NOONE: I can't imagine Sarah not being with us and at the moment don't think there's any need to think otherwise.

PETER OVERTON: There are not private times when you actually wonder about her future?

ROSEMARY NOONE: I do, but I think probably it's a safety mechanism that I have inside that doesn't let me go very far ahead.

PETER OVERTON: Paul and Sarah and others like them may or may not hold the key to how our personalities are formed. Whatever the case though, we could all learn a thing or two from the Willies.

GERALDINE NOTMAN: In some ways, the Willies people have got the better side of life.

PETER OVERTON: It's been called a beautiful mystery.

GERALDINE NOTMAN: He's more than a beautiful mystery, aren't you, mate?

PAUL NOTMAN: Yeah.