Williams Syndrome

Once you get started looking at how things normally develop, you find eye popping examples of not normal. Most are horrifying. Nightmare inducing.

Williams Syndrome is different.

Only about 25 malformed or missing genes stand between health and illness in Williams. That is 083% of the genes. It doesn't look like much .083%. Maybe a rounding error. But that tiny misquote in a string 30,000 genes long means:

Williams people talk a lot, and they talk with pretty much anyone. They appear to truly lack social fear. Indeed, functional brain scans have shown that the brain’s main fear processor, the amygdala, which in most of us shows heightened activity when we see angry or worried faces, shows no reaction when a person with Williams views such faces. It’s as if they see all faces as friendly.

There are other impariments. Difficulty with math and spacial relationships. And most markedly, imperviousness to social cues. Or Dobbs puts it:

They know no strangers but can claim few friends

.

How can 20-25 genes control such complicated behaviors without causing catastrophic cognitive failures? Does this mean a lot of what we think of as "culture" is actually hard wired?

The brains of people with Williams are on average 15 percent smaller than normal, and almost all this size reduction comes from underdeveloped dorsal regions (along the back and the top of the brain) . Ventral regions (at the front and bottom), meanwhile, are close to normal and in some areas — auditory processing, for example — are unusually rich in synaptic connections

The genes that are screwy in Williams seem to have an important structural role, but one with some room for error. These are not the genes that close up the neural tube (see anencephaly if you dare) or that enforce bilateral symmetry.

These genes retard neural development in one part of the brain and in response synapses that are better formed get more action and grow stronger and more durable.

In Williams folks that means language becomes radically more important. And what atrophies?

Generating and detecting deception and veiled meaning requires not just the recognition that people can be bad but a certain level of cognitive power that people with Williams typically lack. In particular it requires what psychologists call “theory of mind,” which is a clear concept of what another person is thinking and the recognition that the other person a) may see the world differently than you do and b) may actually be thinking something different from what he’s saying. Cognitive scientists argue over whether people with Williams have theory of mind.

They can't see guile. Or manners. Or behavioral conventions where we embed much of the meaning we leave out of speech. Sounds lonely.