Describes the Williams syndrome, a rare human genetic disorder that
causes retardation yet leaves language and social skills strangely
unscathed. Syndrome as the opposite of autism; Skills and abilities of a
person with Williams syndrome.
By
PT Staff, published on September 01, 1995
'Bill" has an IQ of 49, a score exports label "moderate
mentalretardation." Yet when asked to describe an elephant, Bill's answer
is complex, almost poetic: "It has long gray ears, fan ears, ears that
can blow in the wind."
Bill's articulate response isn't unusual--for people with Williams
syndrome, a rare genetic disorder that causes retardation yet leaves
language and social skills strangely unscathed. But Williams syndrome is
more than a neurological curiosity. The disorder may reveal surprising
clues to how our brains are organized,says Ursula Bellugi, director of
the Salk Institute's Cognitive Neuroscience lab.
Discovered only in 1961, Williams syndrome (WS) afflicts about one
in 25,000 children in the U.S. In a sense the syndrome is the opposite of
autism: Those who have it are social savants. While autistic kids often
seem to inhabit a private universe, oblivious to the thoughts and
feelings of others, people with WS "are social to the extent that parents
have to stop them from talking to strangers," Bellugi says. So friendly
and well-spoken are they that some wags refer to the Condition as
"cocktail-party syndrome."
The language skills of WS subjects far exceed those of others with
the same IQ--and may surpass even normal controls. A sampling:
o People with Williams syndrome pick up new and unusual words with
ease. Asked to name as many animals as they can in 60 seconds, WS
subjects typically come up with as many responses as normal controls--and
they're more likely to name uncommon animals like weasel or yak.
o They have no trouble grasping unconventional sentence structures,
like passive voice, that overwhelm people with Down's syndrome and other
forms of retardation.
o Natural storytellers, they pepper their tales with sound effects
and with audience hookers like guess what happened next and lo and
behold!
How do these islands of intact skills survive? The neural circuitry
that controls them may be distinct from the rest of the brain, isolated
in a "social module," Bellugi and her colleagues argue in the Journal of
Cognitive Neuroscience (Vol. 7, No. 2). If so, language and social
interaction skills may actually be hardwired into our brains.
Those behaviors are controlled by the frontal cortex and
neocerebellum, regions that "have greatly enlarged in the change from
primate to man," notes Bellugi. It is those brain areas that, in effect,
separate us from lower animals. So our superior language skills aren't
simply an outgrowth of our intelligence, but may actually be the essence
of what makes us human.
PHOTO (COLOR): A boy.
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Williams Syndrome
