Williams Syndrome - evidence for a discrete social brain

David Dobbs offers a very well written essay (PDF here) on Williams Syndrome in the Sunday New York Times Magazine (7/8/07). The syndrome is caused by a well-defined deletion in chromosome 7 that occasionally occurs during the synthesis of egg or sperm cells. Patients have a low IQ (~60) and compromised spatial skill and analytical thought, but are hyper-sociable and friendly, very talkative. An pathway from the orbitofrontal (OFC) cortex to the amygdala that usually signals dangerous or angry faces is inactive; but curiously the OFC-amygdala connection still works normally for nonsocial threats such as pictures of snakes, sharks or car crashes. The existence of this syndrome provides perhaps the strongest evidence for genetically and developmentally distinct class of 'social brain' mechanisms distinct from other higher sensory, motor, and analytical skills.