Keeping Up With The Williamses

4 comments July 8th, 2007at 06:21pm Posted by Eli

NYT Magazine has a fascinating feature about a genetic condition called Williams syndrome, which results in unusual gregariousness and verboseness, and a complete lack of “social fear,” in addition to some cognitive and physical impairments (I apologize for the length; there was an awful lot of juicy stuff to try to boil down):

If a person suffers the small genetic accident that creates Williams syndrome, he’ll live with not only some fairly conventional cognitive deficits, like trouble with space and numbers, but also a strange set of traits that researchers call the Williams social phenotype or, less formally, the “Williams personality”: a love of company and conversation combined, often awkwardly, with a poor understanding of social dynamics and a lack of social inhibition. The combination creates some memorable encounters. Oliver Sacks, the neurologist and author, once watched as a particularly charming 8-year-old Williams girl, who was visiting Sacks at his hotel, took a garrulous detour into a wedding ceremony. “I’m afraid she disrupted the flow of this wedding,” Sacks told me. “She also mistook the bride’s mother for the bride. That was an awkward moment. But it very much pleased the mother.”

Another Williams encounter: The mother of twin Williams boys in their late teens opened her door to find on her stoop a leather-clad biker, motorcycle parked at the curb, asking for her sons. The boys had made the biker’s acquaintance via C.B. radio and invited him to come by, but they forgot to tell Mom. The biker visited for a spell. Fascinated with how the twins talked about their condition, the biker asked them to speak at his motorcycle club’s next meeting. They did. They told the group of the genetic accident underlying Williams, the heart and vascular problems that eventually kill many who have it, their intense enjoyment of talk, music and story, their frustration in trying to make friends, the slights and cruelties they suffered growing up, their difficulty understanding the world. When they finished, most of the bikers were in tears.

(…)

The low I.Q., however, ignores two traits that define Williams more distinctly than do its deficits: an exuberant gregariousness and near-normal language skills. Williams people talk a lot, and they talk with pretty much anyone. They appear to truly lack social fear. Indeed, functional brain scans have shown that the brain’s main fear processor, the amygdala, which in most of us shows heightened activity when we see angry or worried faces, shows no reaction when a person with Williams views such faces. It’s as if they see all faces as friendly.

People with Williams tend to lack not just social fear but also social savvy. Lost on them are many meanings, machinations, ideas and intentions that most of us infer from facial expression, body language, context and stock phrasings. If you’re talking with someone with Williams syndrome and look at your watch and say: “Oh, my, look at the time! Well it’s been awfully nice talking with you . . . ,” your conversational partner may well smile brightly, agree that “this is nice” and ask if you’ve ever gone to Disney World. Because of this — and because many of us feel uneasy with people with cognitive disorders, or for that matter with anyone profoundly unlike us — people with Williams can have trouble deepening relationships. This saddens and frustrates them. They know no strangers but can claim few friends.

(…)

[Williams] is “an experiment of nature,” as the title of one paper puts it, perfect for studying not just how genes create intelligence and sociability but also how our powers of thought combine with our desire to bond to create complex social behavior — a huge arena of interaction that largely determines our fates.

(That seems a rather… callous way of putting it.)

[Ursula] Bellugi, who specializes in the neurobiology of language, was drawn to the linguistic strength that many Williamses displayed in the face of serious cognitive problems. The first person with Williams she met, in fact, came by referral from the linguist Noam Chomsky.

“The mother of that Williams teenager later connected me with two more, both in their teens,” Bellugi said. “I didn’t have to talk to them long to realize something special was going on. Here they had these great cognitive deficits. Yet they spoke with the most ardent and delightful animation and color.”

To understand this uneven cognitive profile, Bellugi gave an array of language and cognitive tests to three groups: Williams children and teenagers, Down syndrome kids with similar I.Q.’s and developmentally average peers. “We would do these warm-up interviews to get to know them, ask about their families,” said Bellugi, who, less than five feet tall and with a ready smile and an animated manner, is somewhat elfin and engagingly gregarious herself. “Only, the Williams kids would turn the tables. They’d tell you how pretty you look or ask, ‘Do you like opera?’ They would ornament their answers in a way other kids didn’t. For instance, you’d ask an adolescent, ‘What if you were a bird?’ The Down kids said things like: ‘I’m not a bird. I don’t fly.’ The Williams teens would say: ‘Good question! I’d fly through the air being free. If I saw a boy I’d land on his head and chirp.’ ”

…Having long studied the human capacity for language and its biological basis, Bellugi assumed that some extraordinary urge to use language drove this hypersociability: “The language just seemed to be erupting out of them.”

Then she attended a meeting of Williams families that included infants and toddlers. “That was about a year into my research project,” she says. “The room was full of little ones — babies, toddlers who weren’t speaking yet. And when I came in the room all the young children old enough to walk ran to the door to greet me. No clinging to Mom; they just broke away. And when I would talk to mothers holding infants — literally babes in arms — some of these babies would almost dive out of their mothers’ arms to meet me.

“I knew then I was wrong. The language wasn’t driving the sociability. If anything, it was the other way around.”

The article then takes a very interesting digression into the connection between sociability and cognition:

For 40 years or so, primatologists like Jane Goodall, Frans de Waal and Robert Sapolsky have been studying social behavior in chimps, gorillas, macaques, bonobos and baboons. Over the past decade that work has led to a unifying theory that explains not only a huge range of behavior but also why our brains are so big and what their most essential work is. The theory, called the Machiavellian-intelligence or social-brain theory, holds that we rise from a lineage in which both individual and group success hinge on balancing the need to work with others with the need to hold our own — or better — amid the nested groups and subgroups we are part of.

(…)

…Robin Dunbar, an evolutionary psychologist and social-brain theorist, and others have documented correlations between brain size and social-group size in many primate species. The bigger an animal’s typical group size (20 or so for macaques, for instance, 50 or so for chimps), the larger the percentage of brain devoted to neocortex, the thin but critical outer layer that accounts for most of a primate’s cognitive abilities. In most mammals the neocortex accounts for 30 percent to 40 percent of brain volume. In the highly social primates it occupies about 50 percent to 65 percent. In humans, it’s 80 percent.

According to Dunbar, no such strong correlation exists between neocortex size and tasks like hunting, navigating or creating shelter. Understanding one another, it seems, is our greatest cognitive challenge. And the only way humans could handle groups of more than 50, Dunbar suggests, was to learn how to talk.

“The conventional view,” Dunbar notes in his book “Grooming, Gossip and the Evolution of Language,” “is that language evolved to enable males to do things like coordinate hunts more effectively. . . . I am suggesting that language evolved to allow us to gossip.”

The article circles back to the Williamses and their inability to perceive social nuances, particularly negative ones like deception and aggression:

…Generating and detecting deception and veiled meaning requires not just the recognition that people can be bad but a certain level of cognitive power that people with Williams typically lack. In particular it requires what psychologists call “theory of mind,” which is a clear concept of what another person is thinking and the recognition that the other person a) may see the world differently than you do and b) may actually be thinking something different from what he’s saying.

(…)

The most significant such finding is a dead connection between the orbitofrontal cortex, an area above the eye sockets and the amygdala, the brain’s fear center. The orbitofrontal cortex (or OFC) is associated with (among other things) prioritizing behavior in social contexts, and earlier studies found that damage to the OFC reduces inhibitions and makes it harder to detect faux pas. The Berman team detected a new contribution to social behavior: They found that while in most people the OFC communicated with the amygdala when viewing threatening faces, the OFC in people with Williams did not. This OFC-amygdala connection worked normally, however, when people with Williams viewed nonsocial threats, like pictures of snakes, sharks or car crashes.

This appears to explain the amygdala’s failure in Williams to fire at the sight of frightening faces and suggests a circuit responsible for Williamses’ lack of social caution. If the results hold up, the researchers will have cleanly defined a circuit evolved specifically to warn of threats from other people. This could account not just for the lack of social fear in Williams, but with it the wariness that can motivate deeper understanding….

As usual, I don’t really have a point here, except that I am always fascinated by just how little it takes (Williams is caused by a flaw in the DNA “zipper” that loses about 25 genes out of 30,000) to knock someone’s brain well outside of the normal, and into an entirely different and even alien way of seeing the world. I’m also amazed at the selectiveness of it – that someone with Williams can recognize scary or threatening nonhuman stimuli, but not human ones.

On the other hand, that selectiveness fits with the social-brain theory: If our brains grew large for the specific purpose of managing social interactions, then it makes sense that those human interactions would be on an entirely different set of circuits from the nonhuman ones.

Do check out the full story, and watch the video of a young woman with Williams telling us about herself.

Entry Filed under: Science

4 Comments

  • 1. Glenn  |  July 8th, 2007 at 8:25 pm

    This syndrome truly describes so many of the people here in New Orleans.

  • 2. ellroon  |  July 8th, 2007 at 9:26 pm

    Wow! That is fascinating. Thanks for catching this, Eli!

  • 3. Eli  |  July 8th, 2007 at 9:31 pm

    My blood could probably be used as an antidote for it.

  • 4. virgotex  |  July 9th, 2007 at 7:27 am

    Thanks for posting this


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