Monday, May 21, 2012

WILLIAMS SYNDROME: A Rare Neurodevelopmental Disorder

What is Williams Syndrome? Originally described by Williams and Beuren in 1961, Williams syndrome (WS) is a rare  neuro-developmental disorder that occurs in about 1 in 8,000 births.


It
affects many parts of the body. Children with this disorder have unique facial features including a short nose with a broad tip or a sunken nasal bridge, puffiness around the eye
, broad forehead, long upper lip length and prominent lower lip, small chin and a full cheeks. Some people who are affected with this disorder have dental problems such as small, widely spaced teeth and teeth that are crooked or missing. In older children and adults, the face appears longer or thin and bony.

This condition is also characterized by mild to moderate intellectual disability or learning problems, unique personality characteristics and cardiovascular problems. Developmental delays are present such as delay of language abilities and delayed motor skill development.  Language abilities are usually observed to be deficient until adolescence period through describing phrases and identifying words including the interaction of speech sounds although a person with Williams syndrome sometimes good in vocabulary.  Development of motor skills are also delayed such as in learning to walk, writing and drawing, assembling puzzles, performing strength and readiness of the arms and hands in physical activity. In addition to this, Attention Deficit Disorder (ADD), anxiety and phobias are common among people with this developmental disorder.


People who are affected with Williams syndrome typically achieve a below-average height and weight. They have developed joint problems, weakened muscle tone and problems in visual, digestive and urinary that probably the reason why they are typically limited in their ability to live independently. But I would like to emphasize that this developmental impairment is attributed more on psychological symptoms than physiological problems.


Can Williams syndrome be inherited? You don’t have to worry because according to the medical experts,  Williams syndrome is not inherited, but still there is a possibility to occur in people even with no history of the disorder in their family.


There is no cure for Williams syndrome. 

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