Thursday, November 29, 2012

"A Hypermusical Species: Williams Syndrome"

Source:  Sacks, Oliver.  “A Hypermusical Species: Williams Syndrome”.  Musicophilia. Toronto: Vintage Canada, 1999. 353-370.


Oliver Sacks' story of Williams Syndrome and music begins at a camp for individuals with this congenital disorder, which had been created by a group of parents in Massachusetts,  all who had children with the disorder.  They saw that their children needed special education, as they were not "normal" but also not "retarded" in the usual sense.  Individuals with the syndrome have very distinct abilities and disabilities.          

For starters, individuals with Williams Syndrome are very friendly, extremely talkative and fearless of strangers- they seem to have a tremendous need to connect and communicate.  They also love storytelling and have an "unusual command of language", for example using vocabulary such as"canine", "abort", "abrasive", "evacuate" and "solemn" even though their IQs are very low.  Comparatively, Down's Syndrome individuals have similar IQs but not nearly the vocabulary.  On the other hand, those with Williams are severely lacking in visuo-spatial skills and cannot do things such as tie their shoelaces, add numbers or put simple Lego blocks together (which interestingly Down's syndrome individuals with the same IQ could do).

What is most interesting to Sacks and most relevant to our course is that they all have an extreme love for music.  Music is so important to them that it is almost omnipresent in their day.  It is like they are addicted to it.  This does not mean that they are all good at it, as was illustrated by Pamela, who loves to sing, but sings off key.  Yet they all love it!  Two more examples of the specific characters Sacks talks about in this chapter are Gloria and Tim.  Gloria, who as a child exhibited extraordinary musical interest and abilities, became an opera singer.  Her dad notes that even though she has a repertoire of 2000 songs, she cannot add five plus three.  Tim also is a very talented piano player.  Together with three others with Williams syndrome, Gloria and Tim created the Williams Five!

Sacks notes that Williams syndrome seems to be different from autism or from musical savants.  In fact he sees it as almost opposite of autism.  In autism he sees that there is a fixation on inanimate objects and an indifference to emotions, whereas in Williams syndrome it is the opposite- an indifference to the nonhuman elements in their environments and a great emphasis on communication.  Williams syndrome is different from savants because savants play music independently of others while Williams syndrome individuals want to share their love of music.

So how are the brains of William's syndrome individuals different from the norm?  The brains of people with William's syndrome are on average twenty percent smaller and have an unusual shape, since the reduction is exclusively in the occipital and parietal lobes.  The temporal lobes are either the same as a normal brain, or even a bit larger, and specifically the primary auditory cortex is often larger.  This explains why those with Williams have such devastating visuo- spatial impairments, and at the same time have such strong auditory, verbal and musical gifts.  The planum temporale is also larger- this is a structure known to be crucial in perception of both music and speech, including absolute pitch.

What was interesting, was that in brain imaging during music listening in those with Williams, music was seen to be processed in a wider set of neural structures, including the regions of the cerebellum, brain stem, and amygdala.  Seeing activation in the amygdala would explain the highly emotional almost helpless attraction to music.


This is actually the first I have heard about this disorder.  Although this disorder has many physical and health difficulties associated with it, the way Sacks presents the disorder in the story does not lead to my first reaction being one of pity.  In general, Williams individuals seem to be happy and beautiful people, in that they are so ready to communicate.  It seems that with some help and counseling, they are capable of living quite independently, as well, leading relatively normal lives.

What was very interesting for me was to what specific degree the characteristics of the disorder were reflected in the different physical structures of the brain.  All the characteristics matched the variances from a normal brain!  For example, that the amygdala processed music completely explained the strong emotional attachment to music.  Or that the occipital and parietal lobes were small- this completely explains the deficiencies of this disorder!  It's almost like if someone had found this kind of brain, they could predict what the resulting individual would be like.

I also find it so positive that parents created support networks with each other.  There are very few individuals with Williams syndrome- about one in ten thousand.  I could imagine that as a parent it would be very beneficial to have others in the same situation to speak to, for practical and emotional reasons.