A Rare Genetic Disorder and What It Tells Us About Music

Introduction

In this study, I investigate the intersection of the fields of music and neuroscience. This is a widely explored subject, but its incredible depth leaves much to be studied. Music has connections to intelligence, cognition, development, language, emotion, mental disorders, and many more neural processes, however, I focus on the relationship music has with mental disorders and related implications. Studying mental disorders, or pathology, is a very common method in neuroscience, as it simulates otherwise impossible experiments in which brain function is altered with observable results. Specifically, I investigate Williams syndrome, a genetic disorder characterized by increased sociability, cheerfulness, and strong language skills. Unfortunately, the syndrome also causes developmental delay and learning disabilities similar to those of Down Syndrome.  However, the most interesting aspect of this disorder is the perceived dramatic increase in musical interest and ability in those with Williams syndrome.  They also can be treated with music with considerable symptom and anxiety reduction. In this post, I research the implications of Williams syndrome and its symptoms to explore the relationship between emotion and music and, in the process, shed light on a potential treatment for this largely ignored disorder.

The amygdala, part of the brain’s limbic system, controls emotions.

The emotion center of the brain is the amygdala, a part of the limbic center located near the center of the brain in the temporal lobe. Emotions are generally caused by activity in either the sympathetic or parasympathetic nervous system through messages sent by the amygdala, However, cognitive identification of the cause of arousal also has a large effect in the perception of emotion. These stimulations are also caused by the release of hormones (blood-borne signaling molecules) and specific neurotransmitters. Music has a profound effect on emotion. It is generally accepted that the emotional effects of music are one of the primary reasons for humans’ innate desire to listen and create. Music may even stimulate opioid release in the brain in some people, those with Williams syndrome among them. Williams people, as they are sometimes to referred to, provide an unusual link between the two topics of emotion and music. Despite general intellectual disabilities, they have increased empathy, sociability, and cheerful demeanors, in addition to expressive language and unusual musical talent. Their limbic systems are generally preserved from abnormalities, though some studies suggest that their amygdalae are larger in volume.  Another key difference in the brains of Williams people is the increased activation of their amygdala when listening to music, an occurrence not seen in those without Williams disorder. This implies that they are more emotionally tied to the music they listen to, but does not explain why they are more musically talented.

Due to the genetic nature of Williams syndrome, many scientists write it off as untreatable. However, music may be the key to alleviating or even eliminating symptoms. Musical therapy has been found to reduce anxiety felt by Williams people, which would likely reduce the negative effects of their abnormal emotions, which include fear of exclusion, feelings of loneliness, and dramatic efforts to be included. Music is certainly beneficial, as many Williams people view it as a means of self expression. Creative therapy (a form of therapy that includes music therapy) has also been researched for other disorders and diseases, such as schizophrenia, bipolar disorder, and Alzheimer’s. A clearer understanding of how music aids the subjects of these disorders will contribute to better treatments and further knowledge about the disorders. This study will hopefully clarify how music is able to be used as a treatment to finally aid the largely ignored Williams people.

Music and Emotion

            Williams people struggle with many tasks, including arithmetic, reading, writing, and drawing (Lenhoff, Wang, Greenberg, & Bellugi, 1997). They generally have low IQs and are classified as having some level of intellectual disability.

A drawing of an elephant by a child with Williams Syndrome

However, they are blessed with a unique musical ability. This ability is expressed very specifically and is not to be generalized. Initially, Williams people were believed to possess exceptional musical ability, expressed through an overall talent for all aspects of music, including pitch discrimination, rhythm, and the playing of instruments (Lenhoff et al. 1997). However, as the disorder was studied further, the realization was made that Williams people are not exceptional, rather, their musical abilities are on par with peers within their mental age as measured by vocabulary (Schellenberg, Don, & Rourk 1999). This is true but for one feature: musical expressiveness (Hopyan, Maureen, Weksberg, & Cytrynbaum, 2001). This was a surprising finding, as the isolation of this single trait of musical ability was something not seen before. Despite the studies, however, many still suspect a deep level of connection between musical talent and Williams syndrome. A living example of this strange relationship is Gloria Lenhoff, a musical savant and Williams person. Lenhoff is a world renowned opera singer, having performed with “such diverse groups as the San Diego Master Chorale and members of Aerosmith,” and can reportedly “sing nearly 2,500 songs in more than 25 language… in a perfect accent” (Maher, 2001). In another example, a boy with Williams syndrome was able to quickly learn to play a 7/4 rhythm with one hand while playing a 4/4 rhythm in the other, an incredibly difficult task (Maher, 2001).

Additional studies were conducted to examine why and how Williams people were able to so well express themselves through music and take such strong expression from music. Lense, Gordon, Key, and Dykens (2014) examined this phenomenon by conducting EEG scans on Williams people while having them evaluate the emotion of a happy, sad, or neutral musical work. They found that not only did the Williams people express a much greater emotional attachment and interpretation of the piece, their brains also showed activation of different neural networks than the control. The researchers also observed that only the Williams people had an increase in neural activity when happy music was played vs. sad, and that only they showed significant differences in the interpretations of facial expressions after exposure to sad or happy music. Ultimately, the researchers suggest that there is a fundamental integration of the standard emotional response to music within other neural networks in Williams people. The EEG recorder gamma activity, a result associated with neural integration or the recruitment of multiple senses. Williams people’s music response networks may be integrated with their social and visual networks, as demonstrated by the findings related to facial processing. Another observation was made by the researchers as to explain the low attention spans of Williams people. In contrast to their typical ability to concentrate, the Williams people in the study were able to focus fully on the music and were not distracted. The obvious hypothesis is that Williams people are so easily distracted from tasks because they are very susceptible to auditory stimuli. This hypothesis is supported by the findings of Järvinen, Ng, Crivelli, Woo-Vonhoogenstyn, Lai, Trauner, and Bellugi (2015), who found that Williams people had heightened sensitivity to auditory stimuli, likely caused by decreased habituation. This also gives a potential mechanism to the increased interest in music seen in Williams people. Sounds command their attention. It may be their affinity for music is due to the fact that they simply cannot ignore it, and thus are exposed to it again and again. In a world of unavoidable noise that distracts, invades, and assaults the ears of the hypersensitive, Williams people may find solace in the beautiful arrangement of music. There is, however, another important factor. The emotional attachment to music is neurological. It can be observed through EEG that Williams people listen to music differently than those without the disorder (Lense et al. 2014). Additionally, one of the most significant features of Williams disorder is the significant alteration to emotion and interpretation. Williams people are consistently cheerful and extremely social, and tend to interpret other’s facial expressions as positive rather than negative. When they listen to music, they respond with increased brain activity to happy music, but do not respond more than the average person to sad music (Lense et al. 2014). These facts, taken together, support the theory that Williams people listen to music with their emotions. They likely play music with their emotions too, which could provide an explanation as to why they are so talented when it comes to performing. Emotion is among the most crucial features of performance. From this research, the question arises as to whether Williams people are born both with sensitive audition and with integrated musical pathways that tie music heavily to emotion, or if the sensitive audition gives rise to a rearrangement of the musical network because of repeated exposure and emphasis. This is a difficult question study, especially since music in general continues to puzzle neuroscientists. There is no one music network, as the large variety of music seems to be equaled by an equally large array of musical responses (Hopyan et al. 2001, Lense et al. 2014). However, the current research provides a network of possible connections to help explain this most unusual of disorders.

Therapy

The advance of modern science has resulted in cures for hundreds of diseases and afflictions. However, genetic disorders remain a struggle for scientists, as a practical solution has yet to be found. Instead, with genetic disorders, the focus has been on therapy to improve the lives and potential of those living with genetic disorders. Many people with disorders are still able to lead normal lives with the help of therapy. Therapy is not a treatment, and generally does not result in the absolving of an affliction. However, with proper therapy, many symptoms can be reduced and even eliminated. For this reason, therapies have become a beacon of hope for progress in the field of genetic disorders.

For many, creative therapy, the use of art, dance, music, or other media to express oneself, is a calming outlet of a creative mind. However, others rely on creative therapy to alleviate the symptoms of an otherwise dictating mental disorder. Creative therapy is among the best treatments for many cognitive disorders. A study by Rylatt (2012) found significant improvement in dementia symptoms, including increased pleasure, communication, and engagement, after a creative therapy regimen. I personally analyzed the relationship between the default mode network, a brain region involved in future planning and memory recollection, and creativity, and found a correlation between the connectivity of the network and creativity. Creativity may therefore stimulate the connections of this network and other brain regions, thus reversing the impact of neurodegenerative diseases like Alzheimer’s and dementia.

Music therapy, a form of creative therapy, has proven to be an invaluable tool for Williams people. Music lessons were shown to increase verbal memory in those with Williams syndrome (Dunning, 2015). Additionally, the Williams Syndrome Association recommends music therapy as one of the most effective therapies (Associated Therapies). Despite many promising leads, little research has been conducted specifically evaluating music therapy’s effectiveness in Williams people. However, evidence points to the promising.

Conclusion

A rapid increase in research of Williams disorder has given hope to a group written off as incurable. While the genetic nature of Williams disorder and similar disorders like Down Syndrome makes them difficult problems to solve, for Williams people, a therapy has arisen from what most of them were doing anyway. It may not surprise many people that music is an incredible healer. With its use as a therapy, it is possible to study the deep connection people have with music through the kings of audiophiles, Williams people. If we can understand what makes Williams people so uniquely touched by music, we can uncover truths about the population as a whole. The importance of this is not to be underestimated. Music can be applied as a therapy for a variety of disorders. Additionally, our history with music may travel back to our origins, where it may shed light on our development and evolution. The music/language debate consistently results in findings about ancestors. Most important to the study of music, however, is the reason that we listen to it. There is some unknown aspect that compels us to play, listen and study, a drive that is almost irresistible. With further research, we can find insight into the fundamental origin, structure, and function of our brains.

 

Works Cited

“Additional Therapies.” Additional Therapies. Accessed November 17, 2015. https://williams-syndrome.org/parent/additional-therapies#1.

Dunning, BA, MA Martens, and MK Jungers. 2015. “Music lessons are associated with increased verbal memory in individuals with williams syndrome.” Research in Developmental Disabilities 36 : 565-78.

Ervaniemi, Mari and Minna Huotilainen. 2003. “The Promises of Change‐Related Brain Potentials in Cognitive Neuroscience of Music.” Annals of the New York Academy of Sciences 999 (1): 29-39.

Järvinen, Anna, Rowena Ng, Davide Crivelli, Dirk Neumann, Andrew J. Arnold, Nicholas Woo-Vonhoogenstyn, Philip Lai, Doris Trauner, and Ursula Bellugi. 2015. “Social functioning and autonomic nervous system sensitivity across vocal and musical emotion in williams syndrome and autism spectrum disorder.” Developmental Psychobiology. Accessed November 17, 2015.

Hopyan, Talar, Maureen Dennis, Rosanna Weksberg, and Cheryl Cytrynbaum. 2001. “Music Skills and the Expressive Interpretation of Music in Children with Williams-Beuren Syndrome: Pitch, Rhythm, Melodic imagery, Phrasing, and Musical Affect.” Child Neuropsychology 7 (1): 42-53.

Lenhoff, H.M., P.P. Wang, F. Greenberg, and U. Bellugi. 1997. “Williams Syndrome and the Brain.” Scientific American 277 (6): 6-132.

Lense, MD, RL Gordon, APF Key, and EM Dykens. 2014. “Neural Correlates of Cross-Modal Affective Priming by Music in Williams Syndrome.” Social Cognitive and Affective Neuroscience 9 (4): 529-37.

Levitin, Daniel J. 2006. This is Your Brain on Music: The Science of a Human Obsession. New York, N.Y: Dutton.

Levitin, Daniel J., and Vinod Menon. 2003. “Musical Structure is Processed in “Language” Areas of the Brain: A Possible Role for Brodmann Area 47 in Temporal Coherence.” Neuroimage 20 (4): 2142-52.

Levitin, Daniel J., and Anna K. Tirovolas. 2009. “Current Advances in the Cognitive Neuroscience of Music.” Annals of the New York Academy of Sciences 1156 (1): 211-31.

Maher, Brendan. “Music, the Brain, and Williams Syndrome.” The Scientist, November 26, 2001.

Patel, Aniruddh D. 2003. “Language, Music, Syntax and the Brain.” Nature Neuroscience 6 (7): 674-81.

Rylatt, P. 2012. “The benefits of creative therapy for people with dementia.” Nursing Standard 26 (33) 42-47.

Schellenberg, G. E., Don, A. J., & Rourke, B. P. (1999). Music and language skills of children with williams syndrome. Child Neuropsychology, 5(3), 154-170. doi:10.1076/chin.5.3.154.7337

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