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Mysteries of Williams Syndrome

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TIMES STAFF WRITER

Unlike Down’s syndrome, whose cause is known (an abnormal number of chromosomes), the roots of Williams sydrome are mysterious--although scientists believe the defect is related to a hyper-production of calcium during fetal development. The condition affects 20,000 to 50,000 Americans. Many cases go undiagnosed.

The syndrome was first identified in 1961 by an English physician, Dr. J. C. P. Williams. In 1963, pediatric cardiologist William F. Friedman, now with the UCLA School of Medicine, documented the condition for the New England Journal of Medicine.

“One very positive thing that has happened in the past decade is that parents of Williams syndrome children have formed support organizations,” Friedman says. “Williams syndrome often causes a number of physical problems, including cardiovascular, so it helps these parents to get together and share information.”

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Sally Meersman of La Crescenta is the membership director for the Williams Syndrome Assn., which comprises 1,600 families nationwide.

Some doctors speculate that at least three Williams syndrome characteristics might go hand in hand: hypersensitive hearing, musical aptitude and verbal ability generally more advanced than that of people with Down’s syndrome.

“The fascinating thing about Williams syndrome is that it leaves children at a level that is called mentally retarded, but that deserves to be called something else,” says Ursula Bellugi, a professor at the Salk Institute for Biological Studies in La Jolla.

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University of Illinois psychologist Leon Miller, author of the book “Musical Savants,” found in his research that mentally handicapped people who are musically gifted have one factor in common: absolute pitch.

Although he has not studied Williams syndrome specifically, he observes that one of its most frequent characteristics, acute hearing, could produce absolute pitch.

“The nice thing about music is that it doesn’t have to mean anything,” Miller says. A mentally handicapped person “can pay attention to the structure of music’s sound independent of its meaning. It’s kind of like paying attention to the beauty of poetry’s sound independent of its words.”

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Even so, Miller adds, most such people do not simply regurgitate compositions. “I found that they played (their instruments) with an awful lot of emotion--it wasn’t just a literal repetition.”

Miller says that Gloria Lenhoff’s aptitude for foreign languages might stem from the same resources that enable her to memorize musical pieces with ease. She could “pick up the accent and inflections of a language without really understanding the semantics,” he says. “Reciting a stream of cliches is one thing, but when you get into deeper conversation that takes nuance or sarcasm into account, forget it.”

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