Mon, Mar 28, 2005 page2

• Williams syndrome kids an enigma to specialists

  MYSTERIOUS Children with the rare disorder are often thought to be mentally retarded, but are gifted in some respects. Studying it may give clues about the brain

  • By Wang Hsiao-wen / STAFF REPORTER
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  Eight months after he was born, Liu Guan-you (劉冠佑) was determined to be mentally retarded. But at age eight, the boy has a sharp memory and a silver tongue, which is seen in his imitation of TV commercial sales pitches.

  "One day when I was cooking, he rushed into the kitchen and went into a three-minute spiel about a Teflon non-stick pan," Liu's mother, Lin Rui-lan (林瑞蘭), recalled with chuckles.

  Lin says she knows that her boy will grow up differently with a unique mind.

  Liu is one of the estimated 100 to 150 children in Taiwan, with a rare genetic disorder called Williams syndrome, in which several genes on chromosome 7 are absent. The syndrome leads to a host of physical and developmental problems. These include cardiovascular disease, dental and kidney abnormalities, lower than average IQ and poor visual-spatial skills.

  The children's physical suffering often puts their parents under heavy strain. Like most children with Williams syndrome, Liu suffers from the narrowing of the aorta, the main artery leading from the heart, a condition that may lead to fatally high blood pressure.

  After making the agonizing decision to have Liu go through a risky heart surgery two months after his birth, his mother lived in fear that her baby might die at any time. Only after years of rehabilitation, regular heart check-ups and acupuncture therapy does she feel that her child will endure.

  Despite Liu's developmental deficiencies, his mother has realized the syndrome's silver lining.

  "There are so many things you want to embrace in the syndrome. Liu is articulate, social, and adored by every one around him," said his mother.

  Liu, doctors said, has an "elf-like" face with a "cocktail party" personality.

  "Williams syndrome children are characterized by a seemingly cheerful facial appearance. They have narrow faces with broad foreheads, depressed nasal bridges, a large mouth, thick lips, and sharp chins," said Tsai Lee-ping (蔡立平), a pediatric geneticist at Taipei City Hospital. "Their eyes are spaced far apart and often have a star-shaped pattern in the iris. This is why people call them `pixie people' and stare at them in public."

  Neurologists and psychologists are also fascinated by children with Williams syndrome, as they offer insights into the workings of the brain.

  "Williams syndrome turns our notions of intelligence on its head," said Academia Sinica vice president Ovid Tzeng (曾志朗), who studies the linguistic, spatial and other cognitive abilities of children with Williams syndrome.

  According to Tseng, people tend to think of intelligence as a single measurable quantity, for example, by an IQ test. People with Williams syndrome nonetheless can display intelligence in certain areas such as language, music, and interpersonal relations. But their IQ averages are typically between 50 and 70, low enough to conclude that they are moderately to mildly retarded.

  It is this uncanny mix of metal peaks and valleys that has fascinated psychologists and neurologists.

  "So far, we do not know the connection between the genetic disorder and cognitive ability. If we can understand Williams syndrome, we will be able to take a giant step toward understanding normal mental processes," Tseng said.

  Tseng, like many other doctors, is charmed by these children's natural ease and openness with strangers.

  "It is hard to teach him not to approach strangers. Liu often puts on a big smile and hugs total strangers," Lin said.

  Another mother of a child with Williams syndrome, Lin Yueh-chen (林月珍), echoed this sentiment.

  "My child Hsuan-yong (宣永) always goes up a complete stranger and says, `can I hug you?'" Lin Yueh-chen said.

  But educating these children remains a vexing problem. Although they are eloquent speakers, children with the disorder often do not understand the meaning of what people say.

  Their facility with language, however, does not extend their spatial abilities, which are impaired.

  "When asked to draw a bicycle, children with the syndrome often draw the components of the bike all over the place," Tseng said. "All the parts are there, but they're not in the correct relationship to each other."

  As Tseng puts it, these children can't see the forest for the trees.

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