Williams Syndrome: It's Not a Fairy Tale
JACKI LYDEN, host:
This is ALL THINGS CONSIDERED from NPR News. I'm Jacki Lyden. Coming up, a novel about the Spanish queen known as Juana the Mad. But first, consider pixies, elves and the wee folk of legends, characters with playful natures, given to singing or dancing.
Some scientists now believe that these creatures could be based on real people, people with a rare genetic disorder called Williams Syndrome. Rhitu Chatterjee recently met Jeremy Vest, a drummer who has the syndrome.
RHITU CHATTERJEE: Even before Jeremy Vest could talk, his mother Sue says he was moved to tears by the Brahms lullaby.
(Soundbite of Brahms lullaby)
Ms. SUE VEST (Mother of Jeremy Vest): When he started to talk he was about a year and a half and he said, you know, that music makes me so sad. Because we would put it on to help him go to sleep, only he would cry and we didn't know until he was old enough to speak.
CHATTERJEE: Now Jeremy's 20 years old and music is still a big part of his life.
(Soundbite of drums)
CHATTERJEE: That's Jeremy on the drums. He's playing with a band called State Radio at the 9:30 Club in Washington, D.C. When he's not performing or practicing, Jeremy's often listening to music.
Mr. JEREMY VEST (Williams Syndrome Patient): I was just upstairs in my room listening to Malaguena from a really awesome show called Blast.
(Soundbite of music)
Mr. VEST: I mean, it's mind-boggling how they can do that kind of thing. They blindfold themselves, they're flipping ...
CHATTERJEE: Jeremy has the small elfin features typical of the syndrome and is a good five inches shorter than his 16-year-old brother Jesse.
We met at his parents' house in Gaithersburg, Maryland.
Mr. VEST: So this is my drum set. (Unintelligible) I can tell you about it. This is a Premier drum set and I have four cymbals, a splash, a...
CHATTERJEE: When Jeremy was little, his parents knew he was very different from other children. He didn't start walking until he was 18 months old. Later he would go up to strangers and start talking, and yet other things, like playgrounds, frightened him.
Ms. VEST: He couldn't get on the slide. He wouldn't do anything that was spinning. I mean, things were scary for him.
CHATTERJEE: Williams Syndrome is caused by a set of genes that go missing. Scientists have slowly begun to understand how these missing genes affect the brains of people with this disorder.
Daniel Levitan is a neuroscientist at McGill University in Montreal and he says that people with Williams Syndrome tend to be more sensitive to music than people without.
Professor DANIEL LEVITAN (McGill University): They'll spend more hours per day listening. When they do listen, they get more emotionally involved. And if they listen to happy music, it tends to keep them happy afterwards for a longer period of time, and similarly, sad music keeps them sad longer.
CHATTERJEE: Music is often the only thing that absorbs their attention. They score low on IQ tests and get distracted easily, but they do love to chat. Jeremy sits at the dining room table constantly moving his feet, wringing his hands and drumming with his fingers. Suddenly he turns to my microphone and starts telling me about his trip back from California.
Mr. VEST: My flight was pretty good.
Unidentified Man: Okay.
Mr. VEST: I'd like to thank Southwest for all their great pilots and their flight attendants for being so caring to me when I'm on their planes. Is that going to be also on the thing too?
CHATTERJEE: What he really wants me to do is hear him play the drums with his brother on the guitar.
(Soundbite of music)
CHATTERJEE: His mother, Sue, says it's remarkable that Jeremy can play the drums and the piano. With so many other things, he struggles to use his hands.
Ms. VEST: And even today, if you watch other little children pick up a Cheerio with the pincer grip, and Jeremy would scoop them off the table because he couldn't use that grip. His handwriting is barely legible.
CHATTERJEE: How Jeremy's hands can produce complicated rhythms and yet fail to sign his name is something neuroscientists don't understand. But Levitan says this is typical for people Williams Syndrome.
Professor LEVITAN: Many of them can't button a sweater or even navigate a stairway, and yet these same individuals can execute the quite precise hand movements needed to play the piano or the clarinet.
CHATTERJEE: Levitan scanned the brains of people with and without the syndrome. In typical brains, only certain parts responded to music. But in Williams brains, there were musical circuits in many areas.
Professor LEVITAN: It's like walking into a house where the walls aren't in the places you expect them to be and the plumbing's not really where you would expect it to be. Maybe the sink isn't next to the shower; it's in a different room or something. It's more like that.
CHATTERJEE: Levitan also found that the amygdala, a tiny part of the brain that controls some of our emotional reactions, is also more active in people with Williams. This could explain why people with the syndrome find music so emotionally powerful and fulfilling.
Mr. VEST: It makes me feel special to have Williams, you know? I feel really happy that I have it, because if I didn't have it, then what would I be like? That's what I keep thinking to myself. Would I be different to other people? Would I have friends like these people, or what would I be doing? I really don't know.
CHATTERJEE: Jeremy graduated from high school last year and is attending the Berkshire Hills Music Academy in South Hadley, Massachusetts, where he's working on music and life skills. Jeremy hopes that someday he will be a professional musician. For NPR News, this is Rhitu Chatterjee.
(Soundbite of music)
Unidentified Man #1: That's Jeremy Vest on the drums...
(Soundbite of crowd cheering)
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