Iconic Symptoms Of Smith-Magenis Syndrome

Delayed Speech And Language Skills

Over three-fourths of all individuals diagnosed with Smith-Magenis syndrome will experience some form of difficulty with speech and language, including delays. During the first year of life, an affected individual typically experiences problems with feeding and swallowing, which can be attributed to the malfunction of the oral motor mechanisms. Affected individuals have poor sucking abilities that make it a challenge to move from liquid food to other soft food textures. A Smith-Magenis syndrome patient can be very delayed in the development of expressive speech skills, and the verbal sounds or speech they do produce is not intelligible. Most patients do not begin to develop sufficient verbal speech skills until the early years of school age. The use of an alternate system to communicate like a picture system, sign language, or gestures can help bridge communication development with the formation of actual speech skills. This method helps reduce the frustration that often becomes a challenge in affected individuals and their loved ones.

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Repeated Self-Hugging

A Smith-Magenis syndrome patient may exhibit the abnormal behavior of repeated self-hugging. The repeated self hugging behavior is described as a tic an affected individual may struggle to control or is involuntary. This tic of the individual's upper body occurs in response to feelings of overstimulation, overwhelming happiness, or excitement. It is not uncommon for Smith-Magenis syndrome patients to produce facial grimaces as they exhibit this repetitive self hugging behavior. Self-hugging describes when an individual clasps both of their hands together tightly and pulls their arms to their sides, or when they tightly cross both of their arms across the chest and squeeze their upper body. Because this behavioral abnormality is one that occurs in response to happiness or pleasure in an individual with Smith-Magenis syndrome, it is considered to be one of the more benign symptoms.

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