The Williams Syndrome: The Unusually Gregarious Brain

The Willams Syndrome: A rare disorder of diminished social fear and little social savvy. Rare condition, helpful video, with genetic and, get this, bowel implications. It looks on the surface like ADD, and involves the prefrontal cortex.

Must read: In today's New York Times, by David Dobbs: The Gregarious Brain – discusses the clinical presentation, the genetics, the social implications and the interesting gastrointestinal presentation of-

Williams syndrome, ….with fairly conventional cognitive deficits, -like trouble with space and numbers, but also a strange set of traits that researchers call the Williams social phenotype or, less formally, the “Williams personality”: a love of company and conversation combined, often awkwardly, with a poor understanding of social dynamics and a lack of social inhibition.

Further,

-The low I.Q., however, ignores two traits that define Williams more distinctly than do its deficits: an exuberant gregariousness and near-normal language skills. Williams people talk a lot, and they talk with pretty much anyone. They appear to truly lack social fear.

The video on the front page of the article is very intriguing for several reasons, not the least of which is the excellent demonstration of a delightful adolescent who suffers with Willams, and clearly demonstrates …her interesting dental structure.

Her teeth look like a symptom of nutritional deficiency to me – iodine deficiency comes to mind. Those interested in child development, and the implications of bowel and brain function will appreciate this next note [pg 4]:

The [Willams] gaze can seem like a hard-wired expression of a Williams’s desire to connect. Yet the gaze can also be seen as a skill learned [?] at the end of the horrible colic that many Williams infants suffer during their first year and before they start to talk well. This window is longer than that for most infants, as Williams children, oddly, start talking a year or so later than most children. It’s during this window that the gaze is at its most intense. Until she was 9 months old, for instance, Nicki Hornbaker [see her video] rarely slept more than an hour at a time, and when she was quiet she tended to look vaguely at her mother’s hairline. Then her colic stopped, she started sleeping and “almost overnight,” her mother told me, “she became a happy, delightful, extremely social child, and she couldn’t get enough eye contact.

-And subsequent to these GI problems she now suffers with a brain development problem with interesting theory of mind implications [more later on mirror neurons]. It sounds more than "genetic" to me. What do you think? Please drop a comment below if you have any references or further reading on these observations.

Stay tuned for an interesting interview coming [likely next week] to CorePsychPodcast with my friend and colleague Dr Tom O'Bryan in Chicago, an expert on Celiac, gluten sensitivity, GI disorders and malnutrition in children and adults.

I will look forward to his thoughts on this interesting Williams presentation.