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Williams syndrome and musicality

Williams syndrome (WS) is a rare genetic, neurodevelopmental disorder present at birth, and can affect both males and females. It is characterised by both medical problems, such as cardiovascular problems, abnormal gait, learning disabilities, low IQs (averaging around 55-60), and physical characteristic such as “elfin” features. People with the condition display high levels of sociability and friendliness, and have been shown to have a natural affinity to music. Williams syndrome individuals are known to respond to music in ways that are not seen in the general typical population. For example, some individuals can be so touched by classical music, that it can bring emotions strong enough to bring the individual to tears. However, this does necessarily mean that all of these individuals will be gifted in music. They share more of common love for music, rather than all of them being musically gifted.

Brain Structure
The brains of those with Williams syndrome are on average 20% smaller, compared to average brain sizes. The weight of the Williams syndrome brain is distributed unevenly, with the majority of the weight and volume predominantly at the front of the brain. The decrease of size and weight of the brain in Williams syndrome is specifically at the rear of the brain. This is reflected in the uneven cognitive profile that Williams syndrome individuals present. In fMRI (functional Magnetic Resonance Imaging) studies, Williams syndrome participants displayed more variable and diffuse activations throughout the brain, compared to typical individuals. They showed increased activation in the amygdala and the cerebellum, and so suggesting that that the neural organisation in the brains of Williams syndrome individuals differs from the typical population.

Amygdala
There have been investigations into the activation of different brain areas in Williams syndrome individuals. When looking at the amygdala’s activation response to music, it was found that there was a significantly greater activation on the right amygdala for the Williams syndrome group compared to the control group. However, there were no significant differences in the left hemisphere. The amygdala is an area of the brain that is known to be associated with emotional processing. The activation of the amygdala could be linked to their strong attraction to music and emotional reactions to music.

Temporal lobes
The temporal lobes are either preserved, or larger than average, in Williams syndrome individuals. It has also been found that with Williams syndrome participants, music and noise in are processed more similarly compared to controls. There was reduced activation in the temporal lobes for music compared to the controls. However, when using a more conservative region of interest (ROI) general linear model (GLM) analyses, it is observed that occipital and early visual areas of the William syndrome brain are activated in response to music and auditory stimuli. The preserved temporal lobes could also contribute to the strong verbal abilities William syndrome individuals present.

Planum Temporale
The planum temporale is known to be important for the perception of speech and music, as well as perfect pitch. Further brain scans show that Williams syndrome brains display significant changes in the planum temporale. In typical individuals, the left side is larger than the right side, however, in Williams syndrome, the planum temporale is almost symmetrical. The right auditory cortex is activated in typical individuals when processing musical pitch, and melody. The planum temporale in the secondary auditory cortex is activated whenever a sound is produced, and more parts of the brain are activated when a pitch is perceived, compared to sound. Williams syndrome individuals used a wider set of neural structures to perceive music, which in turn causes them to process music differently to typical individuals.

Other brain areas and brain usage
It is found that the cells in the primary auditory cortex in Williams syndrome individuals are larger, and loosely packed, which could indicate that there are higher levels of “connectedness”. Musicians are believed to use both parts of their brains more frequently compared to average individuals. In addition to this, they also have a large verbal ability compared to non-musicians. Williams syndrome individuals emulate this; compared to the general population, and even musicians, Williams syndrome individuals use a broader proportion of their brains when perceiving and responding to music. The areas of widespread include the cerebellum, brain stem, and amygdala, which are not as frequently activated in musicians and the general population.

Neurotrophin nerve growth factor
Neurotrophin nerve growth factor (NGF) levels may contribute to this altered (music) sensory processing. Hyperacusis and other auditory abnormalities in Williams syndrome individuals and typical controls were examined, and it was found that NGF levels were elevated in both groups of children from 2 to 6 years of age. However, individuals with Williams syndrome had high NGF levels from 2 to 20 years of age, around four times as long as the typically developing control group. It was speculated that this extended period of elevated levels of NGF could play a part in the abnormal development of cortical regions, and/ or the white matter tracts that connect them.

Musical Memory
Despite the majority of Williams syndrome individuals having low mean IQs, they have an exceptional memory for songs. Musicians, as expected, have a better musical memory. In addition to this, it is speculated that musicians also have a more enhanced verbal memory compared to the general population. For example, musicians are better at recalling a list of random words compared to non-musicians. This is mirrored in Williams syndrome individuals; many have exceptional recall for lyrics and pieces of music. In addition to this, Williams syndrome individuals have an affinity to remember a very large number of lyrics perfectly, and can also do this in different languages that they do not necessarily speak. A famous example within the Williams syndrome world is Gloria Lenhoff. She can sing in 32 different languages, as well as play the accordion. Many others also have a great memory when it comes to lyrics and music pieces, and can recall these well into the distant future. As well as being able to recall music, Williams syndrome individuals are exceptional at retaining information once they have learned it, despite the difficulty initially posed when attempting to teach them the new information. Once the information is learned, it is retained effectively. Contrary to the abovementioned information, having said that, not all Williams syndrome individuals will display these memory skills.

Musical Therapy
Parents of children with Williams syndrome are recommended to have their child assessed by a music therapist. The child will be assessed tand this will determine whether music therapy will be appropriate for that particular child, and whether this will benefit the child in the long term. Despite this, not all Williams syndrome children may benefit from music therapy, or respond to it as positively as others might do. Depending on the level of experience the child has with education and music, a beneficial interest may appear overtime as the child grows and becomes more familiar with music, or after certain experiences. Therefore, in some cases, it may also be suggested that the child is reassessed at a later age. It has been reported by caregivers and parents that Williams syndrome children seem to use music instinctively in a therapeutic manner themselves to reduce anxiety, and to increase positive affect.