Prader-Willi syndrome

Prader-Willi syndrome
A condition characterized by muscle floppiness (hypotonia), excess appetite that if unchecked leads to obesity, small hands and feet and mental retardation. The syndrome is due to the lack in the child of chromosome region 15q11-13 from their father. The child may have two copies of chromosome region 15q11-13 from their mother (a phenomenon called maternal disomy). Although candidate genes in 15q11-13 have been identified, the exact gene or genes that give rise to the syndrome are not known. There is currently no specific treatment or cure for Prader-Willi syndrome. Parents are advised to limit consumption of high-calorie foods, and to use techniques such as special education, speech therapy, and physical therapy to maximize the child’s potential. In some cases, antidepressant or stimulant medication is tried, which may effect appetite, attention, and behavior. Severe psychiatric illness is common in Prader-Willi adults. Those with psychotic illness have a double maternal copy of 15q11-13, suggesting that genes in this region are important in causing psychotic illness.

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Pra·der-Wil·li syndrome 'prä-dər-'vil-ē- n a genetic disorder characterized by short stature, mental retardation, hypotonia, abnormally small hands and feet, hypogonadism, and uncontrolled appetite leading to extreme obesity
Prader Andrea (1919-2001), and
Willi Heinrich (1900-1971)
Swiss pediatricians. Prader was a professor of pediatrics in Zurich. Her articles and chapters in textbooks dealt with such topics as genetics, growth, endocrinology, and metabolism in children and adolescents. Prader and Willi first described the Prader-Willi syndrome in 1956. Cases of the disorder were not diagnosed in the U.S. until the 1960s.

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a congenital condition in which obesity is associated with mental retardation and small genitalia; diabetes mellitus frequently develops in affected individuals.
A. Prader and H. Willi (20th century), Swiss paediatricians

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Pra·der-Wil·li syndrome (prahґdər vilґe) [A. Prader; Heinrich Willi, Swiss pediatrician, 1900–1971] see under syndrome.

Medical dictionary. 2011.

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