Sunday, March 29, 2009

Musical Behavior in a Neurogenetic Developmental Disorder

Evidence from Williams Syndrome

Daniel J. Levitin

Daniel J. Levitin's paper reviews a series of studies performed to assess the musical abilities and behaviors of individuals with Williams Syndrome- a neurogenetic developmental disorder.

What is Williams Syndrome (WS)?

Williams Syndrome is created by a small genetic accident which occurs during meiosis, when a segment of DNA containing 25 genes is lost. The result of which affects abstract thought, so that many WS have a bad concept of spacial, quantitative, reasoning, attention, eye-hand coorination, and reading abilities. WS people tend to be very talkative, and will talk with everyone; they completely lack a sense of social fear. Functional brain scans have shown that the amygdala in WS people shows no reaction when they see angry or worried faces.

Here is an image taken from the National Institutes of Health website depicting the difference in amydala activity scanned in reaction to threatening scenes and faces in WS participants and controls (http://www.nih.gov/news/pr/jul2005/nimh-10.htm)

A photo showing abnormal regulation of the amygdala in participants               with Williams Syndrome (right) compared to controls               (left). The amygdala activates more for threatening               scenes (bottom), but less for threatening faces (top) Abnormal regulation of the amygdala in participants with Williams Syndrome (right) compared to controls (left). The amygdala activates more for threatening scenes (bottom), but less for threatening faces (top).

What is the connection between WS and music?
Not only do WS people show extreme friendliness and near-normal speaking skills, they also tend to be more engaged in musical activities and musicality than others. Levitin reports in this paper a possible neuroanatomical correlate of this engagement, with increased activation in the right amydala to music and to noise.

Based on Levitin and his team's observations, claims of musicality involve many aspects of music including frequent music listening, music performance (for example: a WS person can be able to play the clarinet despite not knowing how to tie their shows) , a deep emotional engagement with msuic, or an above-average musical memory and sound sensitivities (hyperacusis) involving unusual sensitivity to sound, categorization or labelling of sounds that others can't, or anxiety and fear of sounds that non-WS people do not find aversive.

Levitin says that WS can help us better understand the links between genes, brain, and musical behaviors and that WS hypersociability and lack of social inhibitions might be related to their musicality.

The Studies

1st Step:
Characterizing the musical phenotype in WS
The Questionnaire.
Levitin administered a questionnaire to the caregivers of 130 WS participants, 130 Down syndrome participants, 130 autistic participants, and to 130 normal controls. The questionnaire gathered information about physical variables, interest in music, emotional responses to music, musical training, the amount of time engaged in musical activities, and the age of onset of musical activities.

Individuals with WS showed a significantly younger age of onset of musical interest, spent more time per week listenign and playing, and were reported to experience higher levels of emotion when listening to music.

A prinical components analysis revealed seven underlying orthogonal factors that contributed to the profile obtained from the questionnaire. This can be divided up into 7 factors including content related to musical complexity, reproduction, sensitivity, musical theory and achievement, listening habits, positivity, and emotions.

Study of Neural Correlates of Auditory Perception in WS using fMRI

Levitin hypothesized that he would find differences in brain activation bewteen people with WS and controls, and that WS people would show a wider and more diffuse pattern of activation to music and noise stimuli than controls, and that they would show a greater amygdaloidal activation, indexing their heightened emotional reations to music and noise.

Study was conducted usuing a desensitization program that involved a professionally proudced video introdcution to the fMRI scanning procedure, usuing a child's-eye-view of the facility and a child's narration. This was followed to a visit fo an fMRI simulator in which the participants could become acclimated to the noises and enclosed space. 5 WS participants were recruited for an fMRI study of differential processing of music and noise, and five age- and sex-matched controls.
Participants listened to to excerpts from familiar and unfamiliar classical music, as well as the types of noisy sounds that WS people are often sensitive to, such as fans, motors, and leaf blowers.

Results:

Comparing music to noise, WS individuals showed a significantly lower voxel intensity bilaterally in the superior temporal cortex, middle temporal gyri, and superior temporal sulcus. In a comparison of responses to music-minus-rest vs. noise-minus-rest, control participants showed significantly higher temporal lobe activations to the music than the noise, while the WS participants showed virtually indistinguishable activation levels.

He also observed marked differences between WS patients and controls in the right amydala, with WS patients exhibiting far greater activation intensity in the music-minus-noise contrast. This points to a possible neural basis for the unusal acoustical and musical sensitivities observed in affected individuals. Overall, WS participants showed more variable and diffuse activations throughout the brain, and the showed increased activation in the amygdala and cerebellum.

The test of musical skills:

Rhythmic Production Ability

To test rhthym, Levitin and his team presented 8 WS individuals and 8 mentally age-matched controls with a set of clapped rhythums in increasing complexity. The participant had to clap back the rhythm as accurately as possible. Independent coders, blind to hypothesis, and group membership, analyized audio tapes of the test sessions and scored each trial as correct or incorrect or incorrect but very musical nonetheless.

The results showed that the WS and control participants obtained an equal number of correct trials about 66%. However, WS indiviuduals were three times more likely when incorrect to supply a musically compatible rhythm. This was interpreted as a marker of rhythmic ability or creative rhythmicity among the WS participants.

Melodic Production Ability

Levitin presented 12 WS individuals, 12 chronologically age-matched NCs, and 12 individuals with DS a set of melodies increasing in complexity, to assess their melodic reproduction ability. WS and NC were statistically better at melodic repetition than the DS, and not significantly different from one another. He then presented all participants with a set of melodic fragments and instructed them to complete the melodies. The WS individuals were not as good at melodic completion as the NCs. Thus, WS individuals are better at rhymic production than melodic production.


Sources:

Levitin, Daniel J.  (2005) Musical Behavior in a Neurogenetic Developmental Disorder, Retrieved March 15, 2009 from Daniel J. Levitin's Website: http://www.psych.mcgill.ca/levitin/

Dobbs, David "The Gregarious Brain." The New York Times July 8, 2007. Retrieved on March 15, 2009 from nytimes.com





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