Summary - Oliver Sacks writes about Williams Syndrome (known as Williams-Beuren syndrome in Europe). These children and adults are a fascinating group of individuals who love music and love people. Generally, they have an IQ of less than 60 but are articulate. Sacks talks about one young girl (14) who describes an elephant’s appearance and personality in great narrative detail, but cannot draw an elephant that is recognizable in any way. In his book, Musicophilia, Sacks juxtaposes the writing with the drawing (p. 360), and the contradiction between cognitive skills is glaring. Williams children have difficulties with other tasks such as stacking blocks, adding numbers, the ability to tie their shoes or “judge obstacles and steps” (p. 359).
They are very social and engaging even to strangers, meeting their gazes, grabbing their hands, encouraging them to join in the activity at hand. This sensitivity is also evident in their music. They love music and can spend hours absorbed in it and react very emotionally to it. Their precocity in musical abilities is a common thread although only a few may become professional musicians. Sacks summarizes their personalities, “The three dispositions which are so heightened in people with Williams syndrome – the musical, the narrative, and the social – seem to go together, distinct yet intimately associated elements of the ardent expressive and communicative drive that is absolutely central in Williams syndrome” (p. 365).
It is quite rare to have Williams syndrome (1 in 10,000) and it wasn’t until 1961 that cardiologist, J.C.P Williams wrote about it (p. 365). Williams syndrome children and adults have heart and vessel defects. Another similarity is their facial appearance, which is sometimes described as ‘elfin’ with upturned noses, large eyes, small chins and round eyes. Williams syndrome children and adults have a tiny chromosomal deletion of 15-25 genes on one chromosome – which is “less than a thousandth part of the twenty-five thousand or so genes in the human genome” (p. 367).
Through brain imaging and a small number of autopsy reports, some discoveries have been made about Williams syndrome brains. Their brains are generally about 20% smaller than normal brains and the weight is distributed unevenly with the weight and size predominantly at the front of the brain. This disadvantages the occipital and parietal lobes but benefits the normal or “supernormal” (p. 365) size of the temporal lobes. The primary cortex is larger and “there seemed to be significant changes in the planum temporale – a structure known to be crucial for the perception of both speech and music, as well as for absolute pitch” (p. 366).
Another interesting difference in their brains is where they process “musicality” and emotional responses. In a comparison of Williams syndrome individuals, ‘normal’ individuals and professional musicians, it was discovered that Williams syndrome brains use a “wider set of neural structures to perceive and respond to music, including regions of the cerebellum, brain stem, and amygdala which are scarcely activated at all in normal subjects”. The active amygdala is thought to be responsible for “their almost helpless attraction to music” (p. 366). Some of the chromosomal and brain differences can account for the cognitive talents and problems exhibited by Williams syndrome individuals; however, questions remain that are still being researched. Some unknowns may be answered although some may not.
Reflection – Oliver Sacks opened this chapter by describing the summer camp in Massachusetts that he visited in the summer of 1995. This is the place that he first met Williams syndrome children and young adults, a place where he was immediately accepted and embraced. As he started to describe it, I thought about the fairly recent movie Camp Rock starring the infamous Jonas Brothers and Demi Lovato (at least in the tween world). It was based on the story of a summer camp, just like the real one, where young adults gathered for fun, camaraderie, and music. I thought about how it really was more the same than different, if Camp Rock had been a real summer camp and not full of Hollywood stereotypes and perfect hair. Then again, perhaps the musical ability and passion would have been greater in the real summer camp in Massachusetts. And if you live with a rare condition, it might be hard to find others that can relate to you, share your experiences, your struggles, your triumphs. I imagine, although I can't possibly claim to know, what a summer camp experience of like minds could mean for Williams syndrome individuals, family, and friends.
The stories of these children and adults described by Sacks are charming and engaging and amazing all at the same time. I was struck by the stark contrasts in their cognitive abilities, gifted and problematic at the same time. We are fortunate to live in a time when technology allows us to see how parts of the brain react to situations such as in the musicality-emotional response research. It shows that Williams syndrome brain processing is very special and unique. I recognize the impact of the differences in the brains, but also that a tiny chromosomal deficiency can make such an impact is ‘huge’ in my mind. We are truly complex and sophisticated beings and it is astounding how many babies are born each year without complication. I know that I comment in almost every blog on the marvel of our physiological and psychological being, but how can you not.