Mental retardation syndrome affecting mostly males.
The phenotype is variable, combining gynecomastia, obesity, speech
disability, hypogonadism, and small feet.
X-Linked Mental Retardation Syndromic 6; X-Linked Mental
Retardation with Gynecomastia and Obesity.
Very rare syndrome; X-linked
Probable developmental anomaly of the central nervous system,
dysfunction of the hypothalamic pituitary-gonadal axis, with secondary
rather than primary gonadal insufficiency. Principal features are mental
retardation, obesity, gynecomastia, speech difficulties, emotional lability,
tapering fingers, and small feet. Many other signs have been occasionally
Obesity can make venous access, direct
laryngoscopy and tracheal intubation difficult.
Gedeon A, Mulley J, Turner G: Gene localisation for Wilson-Turner
syndrome (WTS:MIM 309585). Am J Med Genet
Wilson M, Mulley J, Gedeon A, et al: New X-linked syndrome of mental
retardation, gynecomastia and obesity is linked to DXS255. Am J Med Genet