We propose the second International Conference on the Rett Syndrome. This syndrome, first described in 1966, has attracted a great deal of recent interest. This interest is based upon: (1) The recent concensus that it represents a distinct clinical entity. (2) It is more common than previously recognized, with incidence estimated at 1:15,000. (3) It presents a remarkably consistent behavioral phenotype with an undoubted predominantly biological basis. (4) There is indication of a disturbance in brain dopamine metabolism. (5) The syndrome occurs only in females, indicating an as yet undefined abnormality in the X-chromosome. Interest and knowledge about this disorder is advancing at a rapid pace, and an international conference about this topic promises to provide new information of both clinical and basic interest.
| (1986) The Rett syndrome. Am J Med Genet Suppl 1:1-404 |
