Characteristics of Prader-Willi

 

MOTOR DEVELOPMENT is delayed typically one to two years, as are most milestones. For example, walking usually occurs around age two. Poor gross motor performance and balance improve slowly and lag behind normal individuals of the same age.

SPEECH AND LANGUAGE PROBLEMS are common. Cause is unclear, possibly poor muscle tone effecting vocal muscles and/or decrease saliva production. Speech therapy is recommended to relieve frustration associated with non-communication. Though language development is delayed, verbal ability is often a strength. Articulation may remain poor.

AVERAGE I.Q.is around70, with a range from 40 to 105. Typically, functioning is below I.Q.level. Abstract thinking and concepts are weaknesses. Even with a lower I.Q., cleverness in food seeking can exist.

BEHAVIOR PROBLEMS, ranging from stubbornness to violent temper tantrums and increasing with age, usually begin to appear during the preschool years when most are pleasent and cooperative. Intervention therapy can be used to modify severity (e.g.,fluexitine serotonin uptake inhibitors have been particularly beneficial in some cases). True depression and pschotic episodes are reported.

COMPULSIVE EATING AND OBSESSION WITH FOOD begin usually between the ages 2-4, though they may start later. Some learn to eat at fixed times and may refuse certain foods, but the insatiable drive for food persists. Sneaking or stealing extra food is common. In most situations, all sources of food must be kept under lock and key. Avoidance of temptation is helpful.

OBESITY occurs in 95% if there are no environmental controls. An increasing number are being diagnosed before onset of obesity. Whether obese at diagnosis or not, more and more have their weight managed within acceptable levels. Calorie utilization is decreased and diets should offer fewer calories than usual (often 1,000-1,200 per day). Exercise is critical to weight control.

SPORTS ACTIVITIES are limited. Running and jumping can cause joint injuries due to poor muscle strength and poor coordination. Incidence of bone fractures is increased, probably related to osteoporosis and decreased muscle bulk. Adaptive physical exercise is necessary in school. Walking, swimming, and stationary exercise equipment are recommended.

DENTAL PROBLEMS are common and may include soft tooth enamel, thick sticky saliva, poor oral hygiene, sometimes teeth grinding, and infrequently rumination. Orthodontia must take into consideration delayed bone growth and abnormal puberty.

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