Williams Syndrome Offers Fresh Insight into Language

Rare Disorder Offers Fresh Insight into Language
July 10, 2006
http://www.npr.org/templates/story/story.php?storyId=5545504

People with Williams Syndrome are elf-like in appearance.

They also have an average IQ of about 60.

their language skills seemed surprisingly good, given their low intelligence.
The disorder appeared to be the opposite of autism, in which people can have normal IQ, but few language skills.

Their motivation to talk to other people is so strong that it helps them overcome some of their initial problems with language.

To use language fully, people need sophisticated social skills that people with Williams Syndrome don’t have.
One of these is something called theory of mind. It’s the ability to understand the thoughts and feelings of the person we are talking to.

Williams syndrome children: no social fear

Absence of racial, but not gender, stereotyping in Williams syndrome children
Andreia Santos, Andreas Meyer-Lindenberg
Current Biology, 20(7), 13 April 2010, Pages R307–R308
http://www.sciencedirect.com/science/article/pii/S0960982210001442

Stereotypes — often implicit attributions to an individual based on group membership categories such as race, religion, age, gender, or nationality — are ubiquitous in human interactions.
Even three-year old children clearly prefer their own ethnic group and discriminate against individuals of different ethnicities.
While stereotypes may enable rapid behavioural decisions with incomplete information, such biases can lead to conflicts and discrimination, especially because stereotypes can be implicit and automatic, making an understanding of the origin of stereotypes an important scientific and socio-political topic.

An important process invoked by out-groups is social fear.
A unique opportunity to study the contribution of this mechanism to stereotypes is afforded by individuals with the microdeletion disorder Williams syndrome (WS), in which social fear is absent, leading to an unusually friendly, high approachability behaviour, including towards strangers.

Here we show that children with WS lack racial stereotyping, though they retain gender stereotyping, compared to matched typically developing children.
Our data indicate that mechanisms for the emergence of gender versus racial bias are neurogenetically dissociable.
Specifically, because WS is associated with reduced social fear, our data support a role of social fear processing in the emergence of racial, but not gender, stereotyping.

journalistic version:
http://www.npr.org/templates/story/story.php?storyId=126136775

Williams Syndrome

A Genetic Drive To Love, Yet Distanced By Differences
by Alix Spiegel
May 03, 2010
http://www.npr.org/templates/story/story.php?storyId=126396171

A great sense Of Empathy

A Life Without Fear
by Alix Spiegel
April 26, 2010
http://www.npr.org/templates/story/story.php?storyId=126224885

When The ‘Trust Hormone’ Is Out Of Balance
by Alix Spiegel
April 22, 2010
http://www.npr.org/templates/story/story.php?storyId=126141922

And so several years ago, Jessica decided that, Williams be damned, she herself would teach Isabelle to distrust. There were other symptoms of the disorder that Jessica’s perseverance had overcome: Though Williams kids often have severe cognitive problems, Isabelle had learned to read.

http://emedicine.medscape.com/article/893149-clinical
Mar 12, 2012

  • Interest and enthusiasm for music is almost universal in patients with Williams syndrome
  • As many as half of all children with Williams syndrome may exhibit autism spectrum social and communicative deficits.
  • Children with Williams syndrome are described as overly friendly, hyperactive, inattentive, and hypersensitive to loud sounds or certain types of sounds.