July 19, 2007 — -- What if there was a condition that made people intensely happy, yet socially awkward?
It's the question raised by one of the most mysterious phenomena known to science: Williams syndrome, a condition in which the areas of the brain that process hearing are more rich in connections than a normal brain, resulting in people who can socialize and hear music better than others, but have problems dealing with everyday interactions.
While people with Williams syndrome have smaller brains than average, studies have shown that they experience sound — like music and language — more intensely than the rest of the population. They also tend to be incredibly sociable.
"When you interact with a person with Williams syndrome, there's an obviously gregariousness that is so dramatic that you cannot not notice it," said Dr. Marty Levinson, who has treated Williams patients. "It's something about their brain that when music comes in it goes through pathways, it creates reactions and it has an effect on them."
According to Allan Reiss, a neuroscientist at Stanford University's medical school, Williams occurs when there is a loss of about 20 genes on one of the human chromosomes, but it is usually not hereditary.
"It's due to the loss of those genes in either the sperm or the egg," he said, "but it's typically not passed on from father or mother to child, except in rare conditions."
Ben Monkaba, 21, has Williams syndrome. He gravitates toward people, always eager to make them happy.
"Most kids with Williams are the first ones to say to someone, 'Do you need a hug? Do you know what I mean? Are you having a bad day?'" said Ben's mother, Terry Monkaba. "They intuitively know those things."
He also loves to play music, especially on the drums, and elicit reactions from his audience.
"I have [the] ability to make people happy, which is an easy thing to do," Ben Monkaba said. "It's just a pleasure to play music. It's really fun just to watch people and to see their reactions."
For him, music is an escape from the hardships he faces every day. Because of Williams syndrome, he has medical problems and developmental delays. Monkaba has already endured four surgeries to repair damaged heart valves, a common problem for those with Williams.
Abstract thinking and physically navigating the world are not easy for him. Though Monkaba can delight people with music, he can't wrap his head around the seemingly simple task of putting a piece of paper in an envelope.
"To do any kind of tasks that involve his hands and involve sort of putting details together is difficult for him," his mother said.
And with Monkaba's gregarious nature comes an inability to read social cues and boundaries, which can create awkward situations.
"Ben is a very social being, but he's not the best socially. Everyone is friendly to him but he's never had a close friend — or very few — and that's hard for him," Terry Monkaba said.
Some doctors speculate that Williams syndrome may be linked with autism, another disorder characterized by problems with socialization. By studying Monkaba and others with Williams, Reiss thinks doctors could uncover new ways to treat autism and other social conditions.
"Williams might provide a little window of understanding into human social behavior that will help us be a little more creative in designing new treatments for people with autism or disorders like that," he said.
Find out more about Williams syndrome at http://www.williams-syndrome.org/