Sunday, November 16, 2008

Music, the brain and Williams syndrome: rare disorder offers insight into the genetic basis of cognition (focus on Genes and Genomics)

1. Reference
Music, the brain and Williams syndrome: rare disorder offers insight into the genetic basis of cognition (focus on Genes and Genomics)
By Brendan A. Maher
The Scientist
November 26, 2001
For Dr. Lee Bartel – Music and the Brain 2122H
A Summary, Review and Response
Lani Sommers

2. Summary
Williams syndrome is a neurodevelopmental disorder caused by a deletion of about 20 genes of chromosome 7. People with Williams syndrome have characteristics such as pixie-like features, upturned nose, small chin, protrusive ears, stunted growth, heart problems, poor visuospatial cognition, sensitivity to loud noise, gregarious personalities and an average IQ of about 60.

Almost all patients with Williams Syndrome have an extraordinary connection with music. Even though people with Williams Syndrome have short-lived attention spans they will spend hours listening to or making music. There is some research which illustrates a high occurrence of perfect pitch and excellent rhythmic ability among this group of people. For example, one boy with the syndrome was able to tap a 7/4 rhythm with one hand while keeping 4/4 time with the other.

Audrey Don, a neuropsychologist at the Good Samaritan Hospital in Seattle, was one of the first researchers to explore the relationship patients with Williams Syndrome have with music. She administered several musical tests of tones and beats to people with Williams syndrome and found that “musical ability matches verbal ability and was higher than the Williams’ children overall cognitive abilities.” She also found that the patients had very strong emotional connections to music.

Other research done with patients with Williams Syndrome showed that these children did as well as normal musically trained students, however, they were able to offer “creative completion” to some of the test rhythms.

Further to this research, Howard M. Lenhoff, professor emeritus, School of Biological Science, University of California, completed a study linking Williams syndrome to a high occurrence of perfect pitch. This condition normally occurs in one out of 10,000 people in Western populations. In the case of patient’s with William’s syndrome the incidence rises to 1 out of 1000.

Usually, a person has to study music before the age of six in order to develop perfect pitch. Many of the Williams Syndrome subjects started studying music much after this critical period yet have still been able to develop perfect pitch.

In relation to brain studies, research has shown that in patient’s with Williams Syndrome the neocerebellum seems to be connected to their phonological working memory, a kind of “short-term memory for sounds.”

Music for patients with Williams Syndrome seems to be what helps them to have a sense of normalcy and fulfillment in life. There is much to be learned from the connection these children have with music and its affects on their brain function and development.

3. Reflection

The article, “Music, the brain and Williams syndrome: rare disorder offers insight into the genetic basis of cognition,” by Brendan A. Maher was interesting; however, it could have been more informative and specific when making reference to the brain. I felt that all of the ideas about Williams Syndrome, Music and Brain Development were superficially connected and more detail was required. That being said, this article was an effective introduction to the description of Williams Syndrome and the occurrence of music proficiency, including the incidence of perfect pitch in children suffering from the syndrome.

The description of Gloria Lenhoff, the 46 year-old lyric soprano singer who can sing nearly 2500 songs in more than 25 languages in a perfect accent was very effective in setting the tone for the article as an introduction to the disease. It is absolutely amazing that a person can be so incredibly skilful in one aspect of their life can be so deficient in another because of a chromosome deficiency. The fact that 1 in 1000 patients with Williams Syndrome have perfect pitch is an incredible statistic; however, Maher fails to delve into the details of this matter adequately or effectively. He touches on a few musical research projects and studies using MRI to examine patients with Williams Syndrome but the results of these studies seem to be superficial and inconclusive in the context of his paper. The title of the article would indicate that the reader is going to find some hardcore data about Music, the Brain and Williams syndrome, but instead, the reader finds very little concrete evidence about the three topics. Maher gives excellent background information about the syndrome and the affinity the people suffering from the syndrome have for music however; this article is better suited as simply an introduction to the syndrome, rather than a study in regards to music and the brain.


Lee said...

It is thrilling to know some people read our blog entries but it is astounding when the research reviewed and the focus of the article responds. Thanks you Dr.Lenhoff for your email response.

I received the following email from Dr. Lenhoff:
Dear Professor Bartel,

Google alert sent me a paper of one of your students on Williams syndrome and music. In it she mentioned my research on absolute pitch, and also wrote about the phenomenal savant talents of my daughter Gloria.[There are others with savant abilities, it is just that my daughter was ne of the ifrst recognized.

If you would like, I would be pleased to send you a DVD and a few CDs of Gloria performing.

There are so many opportunities for research in this interesting genetic population. Attached is a short bio of Gloria.

Glad that you are teaching that class.

Good wishes,

Howard Lenhoff

Here is Gloria's bio (as well as Dr. Lenhoff's)


Lyric soprano and accordionist Gloria Lenhoff, a Williams syndrome musical savant, has a repertoire of over 3,000 pieces and has performed overseas and throughout the United States. Gloria, who has received acclaim from near and far, sings in 30 foreign languages, providing music for all tastes: popular, religious, folk, classical and opera. Her IQ is 55 and she has an outstanding stage presence.
She first came upon the national scene in the PBS award-winning documentary, “Bravo Gloria,” directed by Arlene Alda. In the past few years Gloria has been featured on 60 Minutes (updated once and broadcast six times), Nightline, Inside Edition, Discovery Health (2006), The Learning Channel (2006) and in four foreign specials: Chile, Holland, and two Japanese channels (2003-04). She has both absolute and relative pitch and learns by listening.
Gloria was the first Williams musician to come upon the international scene and has stimulated the current flurry of activity regarding the uncanny musical abilities of the population of cognitively impaired people having Williams syndrome. Because of her, the first music camp for the cognitively impaired was started near the Tanglewood Music Center in Lenox, MA. The camp ran for ten consecutive years. As an outgrowth of the camp, the new Berkshire Hills Music Academy (BHMA) for the cognitively impaired, a year-round residential academy, the first of its kind, has completed five academic years and now sponsors the summer camp. The BHMA is situated on an estate acquired from Mt. Holyoke College in Massachusetts On October 25, 2006, a book on Gloria’s story was published –The Strangest Song, by Teri Sforza, Prometheus Books.
She has performed with members of the LA Opera and the Boston Lyric Opera, and at the Wheeler Opera House of Aspen, CO and the Bardavon Opera House of NY as well as at the Treasure Island Hotel in Las Vegas, the Ritz Carlton in Boston, and the Beverly Hills Hotel. Ms. Lenhoff has been featured twice as guest soloist with a San Diego Community Orchestra (TICO) and as featured soloist with the San Diego Master Chorale. In the she has performed in England Spain, and Israel. At the Ritz Carlton in Boston she sang and played her accordion with Aerosmith musicians. Three weeks later she soloed at the Kennedy Center in Washington, D. C. With Opera Memphis, she sang Soprano I in the Hebrew and Philistine Choruses in the production of Samson and Dalila (2005), Celebration of Denyse Graves (2006) and Il Trovatore. January of 2007. In April, 2009, she will be in Gounod’s Faust.

Howard M. Lenhoff, Ph. D. Professor Emeritus, University of California; Adjunct Professor of Psychology, University of Massachusetts (2001-4); Adjunct Professor of Biology, University of Mississippi; Principal Investigator of National Science Foundation grant on Music Cognition in Williams Syndrome. Senior author of “Williams Syndrome and the Brain,” Dec., 1997 Scientific American and Absolute Pitch in Williams Syndrome. A Founder, and on Professional Committee of Berkshire Hills Music Academy for Cognitively Impaired (BHMA). Former member of the Board of Trustees of the Williams Syndrome Association and President and Executive Vice President, Williams Syndrome Foundation. Co-editor and chapter author of Williams-Beuren Syndrome: Research and Clinical Implications, Johns Hopkins University Press, 2006. Father of Williams syndrome soprano and accordionist, Gloria. Professor Lenhoff is recognized as a popular teacher at the University of California, known for his enthusiasm, broad knowledge, wit, and ability to make difficult concepts understandable to the non-biologists.

Investigator of the Howard Hughes Medical Institute, 1958-1963
Distinguished Fellow of the Iowa Academy of Science, 1986.
Honorary Member of the Société de Physique et d'Histoire naturelle de Genève 1990.
Phi Beta Kappa, Phi Kappa Phi, Sigma Xi
Thirteen books and over 200 academic papers

Lampy said...
This comment has been removed by the author.
Lampy said...

That's really neat! I'd love to hear Gloria singing - the description about her in the article was really interesting. I impressed that we got a response from her father. He was also one of the researchers referred to in the paper I reviewed. I'm going to see if I can find the documentary on her - it would be great to learn more!

s@bd said...

astounding is right!