Self Conception, Facial Perception, and Social Behavior in People with Williams Syndrome Jocelaine PresenDieu
Columbus State University
This paper explores three aspects of Williams syndrome. Williams syndrome is a rare developmental disorder caused by the microdeletion of genes from chromosome 7q11.23 (Mervis, 2003). The microdeletion is evident in almost all individuals with Williams syndrome so is the personality characteristic. Individuals with Williams syndrome are social, easily distracted, overly friendly, have severe anxiety and have an empathetic nature (Morris & Mervis, 2000). The personality of individuals with Williams syndrome is detected at an early age and consistent through adulthood. The three aspects discussed are facial perception, self conception and social behavior. Each aspect is important for understanding an individual with Williams syndrome in a holistic manner. The evidence presented in the paper all overlap and can be a direct product of individuals with Williams syndrome personality. The evidence presented supports further research in this field. Our hope is that further research continues to be done in order to expound upon the theorized genetic basis. Keywords: Williams syndrome, social behavior, facial perception, self conception
Self Conception, Facial Perception, and Social Behavior in People with Williams Syndrome Williams syndrome is agreed upon by researchers to be a rare developmental disorder coupled with mild to moderate mental deficiency or learning inability (Mervis, 2003). Williams Syndrome only occurs in 1 of every 20,000 births (Morris & Mervis, 2000). The first reports of Williams syndrome came from Great Britain following World War II (Mervis & Morris). Williams syndrome is unique compared to other developmental disorders due to a recurrent pattern of facial characteristics. This disorder has a biological origin. Williams syndrome involve presynaptic cytoskeletal protein (PCLO). PCLO gene leads to chromosome 7q11.23 (Zafeiriou, Ververi, & Vargiami, 2007). Williams syndrome derives by a 1.5 megabase micro erasing of at minimum 16 genes on chromosome 7q11.23. Almost all patients suffering from Williams syndrome have shown a deletion in chromosome 7q11.23 (Mervis & Klein-Tasman, 2000). This evidence has strengthened the theory of its biological connection. Research has shown Williams syndrome tends to effect infants and young children during the developmental stages. The beginning of vocabulary obtainment is delayed in these children. Mervis & Klein-Tasman (2000) cited a study where majority of the participants experienced vocabulary sizes falling in the lowest percentile compared to the norm group. William syndrome sufferers have problems with visuospatial construction. We will discuss visuospatial construction later on. Studies have also discovered young children with Williams syndrome, assume that a distinct word said in the presence of an unfamiliar object refers to the object as a whole, instead of a segment or other characteristic of the object. Sufferers of Williams syndrome do have a heightened ability in auditory rote memory. Auditory rote memory is being able to learn or memorize by repetition but only through auditory means. One can understand all of these points as the groundwork for the present paper. Learning difficulty, visuospatial construction, and auditory rote memory are concurrent patterns viewed in Williams Syndrome patients. As, you continue to read you will notice not only the symptoms and biological basis the same in majority of the patients but also their personalities. Individuals plagued with Williams Syndrome find difficulties with tasks associated with visuospatial construction. Visuospatial construction is the ability to see an object as an assortment of parts and then to construct a duplicate of the original from the parts (Mervis & Klein-Tasman, 2000). Personality
Individuals with Williams...
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