Williams Syndrome and Brain Organization

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Williams Syndrome and Brain Organization

To understand the human brain, looking at behavior as an indicator of brain function has led researchers to focus on individuals with Williams syndrome (1). The limitations as well as highly developed features in behavior found in people with this syndrome have attracted investigators. The unique behavior displayed has led many to believe that it may be possible to unravel brain organization by locating the damaged areas in individuals with Williams syndrome and connect them to the behavior observed in these individuals (1).

Individuals with Williams syndrome are all unique in their behavior, but most share common features. When given IQ tests they tend to receive scores that are below the average (1). The most common feature in is mental retardation, with about 95% with IQs in the mild to moderate range of retardation (3). Along with low IQ scores, these individuals tend to have limited writing and arithmetic skills. Some individuals display developed abilities to compose stories. They seem to be comfortable narrating and manipulating their voice to express their story (3). They are known for their musical talents and spoken language abilities as well as their sociable tendencies (1).

Research on individuals with Williams syndrome has led to many discoveries. One of the biggest puzzle pieces uncovered is the genetic cause of the disorder (4). A small piece missing in one of the chromosome 7 copies was found to be the cause of Williams syndrome (4). Individuals lose about 15 to more genes contained in this missing piece. About 95% of the individuals with Williams syndrome display this deletion (1). Some features of the disorder include problems with the heart, blood vessel...

... middle of paper ...

...a Bellugi. (1997, December). Williams Syndrome and the Brain. Scientific American. 68-73. from http://www.sciamarchive.org

2)Institute of Child Health, Crucial Differences Between Developmental Cognitive Neuroscience and Adult Neuropsychology, by Annette Karmiloff-Smith.

http://www.ich.ucl.ac.uk/ich/html/academicunits/neurocog_dev/aks/Neuropsychology.pdf

3)Center for Research in Language, Williams Syndrome: An Unusual Neuropsychological Profile by Ursula Bellugi, Paul P. Wang, and Terry L. Jernigan.

http://crl.ucsd.edu/courses/commdis/pdf/Bellugi-et-al.pdf

4)National Center for Biotechnology Information, Genes and Disease information

http://www.ncbi.nlm.nih.gov/disease/Williams.html

5)Guardian Unlimited newspaper, Summary of lecture by Annette Karmiloff-Smith.

http://education.guardian.co.uk/higher/medicalscience/partner/story/0,9877,588690,00.html

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