title: Neuropsychology and socioeconomic aspects of Klinefelter syndrome: new developments.
abstract: OBJECTIVE
  To summarize recent important studies on neuropsychology and epidemiology of Klinefelter syndrome. PubMed was searched for 'Klinefelter', 'Klinefelter's' and 'XXY' in titles and abstracts. Relevant studies were obtained and reviewed, as well as other articles selected by the authors.
  
  
  RESULTS
  Klinefelter syndrome is the most common sex-chromosome disorder in humans, affecting one in 660 men. The key findings in Klinefelter syndrome are small testes, hypergonadotropic hypogonadism and cognitive impairment. Klinefelter syndrome scores significantly below education matched controls on a range of cognitive tests with verbal skills displaying the largest effects. Boys with Klinefelter syndrome are often in the need of speech therapy and many suffer from learning disability and may benefit from special education. New studies are elucidating aspects of cognitive functioning and suggesting that neuropsychological treatment may be of value. The socioeconomic status and educational level of Klinefelter syndrome is severely affected with many struggling to achieve any or only shorter education, resulting in low-income levels and early retirement. In addition, few become fathers and fewer live with a partner compared with controls. Medical treatment is mainly testosterone replacement therapy in order to alleviate acute and long-term consequences of hypogonadism, as well as, treating or preventing the frequent comorbidity.
  
  
  CONCLUSIONS
  The neurocognitive phenotype of Klinefelter syndrome is being unraveled and the need for psychological and cognitive treatment in many cases is evident. The neurocognitive deficits no doubt influence the socioeconomic status of many Klinefelter syndrome patients, which is clearly inferior to age-matched controls.
authors.affiliation: aDepartment Clinical Genetics, Aarhus University Hospital bDepartment of Endocrinology and Internal Medicine cCenter of Functionally Integrative Neuroscience dCenter for Semiotics eDepartment of Molecular Medicine, Aarhus University Hospital, Denmark.
authors.forename: Anne
authors.initials: A
authors.lastname: Skakkebæk
authors.forename: Mikkel
authors.initials: M
authors.lastname: Wallentin
authors.forename: Claus H
authors.initials: CH
authors.lastname: Gravholt
mesh_terms: D002648:Child; D006801:Humans; D007713:Klinefelter Syndrome / Q000191:economics / Q000503:physiopathology / Q000523:psychology; D008297:Male