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November 15, 2005
Isolating Excessive Friendliness For the Good of Humankind
Excessive friendliness is one of the indications that people with Williams-Beuren Syndrome (WS) exhibit. Also known as 'elvin face syndrome' because of the general common appearance of upturned noses, wide mouths and small chins, WS is a neurodevelopmental disorder that affects 1 in 7500 people.
WS was first identified the early 1960s in a set of papers that described children with a unique set of facial, cognitive, heart defects and excessive social behavior. Since the discovery in the early 1990s that the syndrome is caused by the deletion of a tiny section of 28 genes on one copy of chromosome 7, researchers have been using imaging, cognitive tests, and genetic analysis to identify the different roles that the genes within the section play in the development and functioning of the brain.
In this week's Science, an excellent review article discusses the progress being made in understanding how these 28 genes, out of the thousands involved in brain development, cause specific aspects of the disorder. The news article "Friendly Faces and Unusual Minds" reviews our increasing understanding of multiple deficits associated with the disorder including: using fMRI to show that WS individuals show significantly lower neuronal activity in a part of the brain used by the spatial processing pathway of the visual system; using MRI scans have revealed structural details of WS-affected brains which revealed different folding patterns in specific areas of the brain; and a multitude of detailed genetic studies to isolate the specific contribution of different genes to the cognitive deficits presented by this disorder.
What I find most interesting about this disorder is the research being carried out on the excessive friendly behavior. Interestingly, while individuals with WS exhibit social fearlessness, they also "display high levels of nonsocial anxiety, such as fear of heights." The article describes a set of experiments that researchers performed using fMRI which showed that WS-affected individuals who were shown threatening faces exhibited a lower level of activation of the amygdala than control groups and a higher activation when shown when shown threatening scenes relative to controls. To date no one has determined the genetic link to these behavioral characteristics.
Last week at the inaugural opening of the MIT McGovern Institute for Brain Research, Nobel Laureate Eric Kandel postulated about a new approach to treating certain brain-related illnesses which I believe may open up an entirely new era of therapeutic development. For example, instead of just studying depression and treating its neurological manifestations, why not look at the opposite end of the emotional spectrum, to something like the neurobiology of joy, and try to accentuate those characteristics as well.
Given this logic perhaps one of the values of studying and understanding extreme friendliness found in WS patients could be a new treatment for those with profound social anxiety. Perhaps, Kandel is on to something.
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