Williams Syndrome and human sociality

There's a great article by David Dobbs in the New York Times Magazine about Williams Syndrome (WMS), a condition with a diverse and remarkable array of cognitive symptoms. I have vivid memories of my first exposure to WMS--watching a documentary hosted by Oliver Sacks for my "Psychology of Music" class. In one of the first scenes, Dr. Sacks introduces himself to a 6-year old girl with WMS, who eagerly and cheerfully insists "Don't be shy, Mr. Sacks." In another scene he takes her to a sandwich shop, where she enthusiastically engages the employees and fellow customers in conversation, offering hugs to all within reach. This behavior exemplifies one of the most remarkable endowments of children with WMS--endearing, socially fearless personalities, marked by extreme gregariousness and emotional empathy.

This charm is facilitated by a peculiarly rich vocabulary and proficiency with language; for example, when asked to name some animals, a WMS child responded "Brontosaurus, tyranadon, brontasaurus rex, dinosaurs, elephant, dog, cat, lion, baby hippopotamus, ibex, whale, bull, yak, zebra, puppy, kitten, tiger, koala, dragon..." quickly and fluidly naming exotic (though occasionally non-existent) animals as if reading them off a list. When striking conversations with strangers, they are extremely loquacious, to the point where they appear to burden the listener with verbosity.

Adding to the list of aptitudes of WMS people is a great affinity for music (hence learning about the condition in my "Psychology of Music" class). People with WMS can have savantlike musical skills, and those without notable musical gifts nevertheless feel "drawn" to music, an inclination likely aided by an acute sensitivity to sound. One scene of the documentary featured WMS children walking through the woods, commenting on how loud the bees and rustling leaves were (sounds which were more or less unnoticed by Sacks).

These remarkable virtuosities with language, social interaction, and music are accompanied, however, by profound cognitive impairments. The average IQ of a person with WMS is in the 60's, and the vast majority cannot live independently. Despite their seeming fluency with verbal communication, people with WMS have poor language comprenension, incapable of understanding the underlying meaning of most conversations. Their communication, though voluminous, lacks depth and subtlety, and rarely goes beyond "small talk."

This intriguing disconnect pervades social interactions beyond spoken language; in spite of their gregariousness, people with WMS often fail to grasp social cues, including facial expression and body language. Moreover, the extreme geniality of WMS people is indicative of an underlying problem: a complete lack of social fear. According to the article, "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."

Children with WMS also have significant deficiencies in spatial processing and dealing with numbers. In another memorable scene from the documentary, Sacks presents the child with a plate of muffins, asking her how many she thought were on the plate. "3," she immediately and eagerly replied. There were clearly over 10. When then asked to make a + shape out of four rectangular pieces, she arranged them haphazardly, seemingly at random, at which point she cheerfully announced "Done!"

WMS thus provides a captivating mélange of cognitive strengths and weaknesses. Unlike most forms of mental retardation, in which most or all cognitive abilities are concurrently impaired, the distinct peaks and valleys of aptitudes in WMS allows a dissociation between specific abilities and "general intelligence." Further, the genetic basis of WMS is known: it arises from a deletion of ~28 known genes from chromosome 7. Thus, WMS offers a tantalizing opportunity to understand the genetic influences on complex brain functions, which I plan to explore in a future post.

This post, however, was inspired by a separate, equally captivating story woven by the WMS condition: the implications for human social behavior. Why, despite their affability and charm, do WMS people find it hopelessly difficult to make friends? According to Dobbs, this paradox "makes clear that while we are innately driven to connect with others, this affiliative drive alone will not win this connection. To bond with others we must show not just charm but sophisticated cognitive skills."

So why is it that all our relationships, even casual friendships, demand intelligence? Why was it so difficult to believe that Jenny would marry Forrest Gump? The article broaches two related and overlapping evolutionary theories, the "social brain" theory and the "Machiavellian-intelligence" theory. These theories propose, respectively, that humans evolved large brains to generate complex social relationships, and that deception and manipulation (and the ability to identify these two behaviors) are necessary to successfully compete amongst other members of society. Thus, as Steven Pinker suggests in The Language Instinct, "human evolution was propelled more by a cognitive arms race among social competitors than by mastery of technology and the physical environment."

Social life presents a convoluted tension, involving (as stated by Ralph Adolphs and quoted by Dobbs), a “complex and dynamic interplay between two opposing factors: on the one hand, groups can provide better security from predators, better mate choice and more reliable food; on the other hand, mates and food are available also to competitors from within the group.” Thus, our survival is contingent on a delicate balance between getting along with others and outperforming them. Requisite for maintaining this balance is a comprehensive understanding of subtle and complicated social dynamics, enabling both manipulation and the detection of manipulation by others. Dobbs writes that:

"People with Williams, however, don’t do this so well. 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.

...it’s clear that Williamses do not generally sniff out the sorts of hidden meanings and intentions that lie behind so much human behavior."

The article concludes with a fascinating question about being human: is our social behavior driven more by the urge to connect or the urge to manipulate the connection? Are we trying to make friends, or do we only care about being genetically more successful than our peers?

"We dominate the planet because we can think abstractly, accumulate and relay knowledge and manipulate the environment and one another. By this light our social behavior rises more from big brains than from big hearts.

...

The disassociation of so many elements in Williams — the cognitive from the connective, social fear from nonsocial fear, the tension between the drive to affiliate and the drive to manipulate — highlights how vital these elements are and, in most of us, how delicately, critically entwined. Yet these splits in Williams also clarify which, of caring and comprehension, offers the more vital contribution. For if Williams confers disadvantage by granting more care than comprehension, reversing this imbalance creates a far more problematic phenotype.

As Robert Sapolsky of the Stanford School of Medicine puts it: “Williams have great interest but little competence. But what about a person who has competence but no warmth, desire or empathy? That’s a sociopath. Sociopaths have great theory of mind. But they couldn’t care less.”"

Link to the NYTM article.