History of Rett Syndrome


Rett syndrome was discovered and named after Dr Andreas Rett. He originally noted that the syndrome was of a progressive nature based on the evidence that a child with the disorder seems to develop normally in the first 6-18 months of development. On April 8, 1983 he organized the first symposium covering Rett syndrome, inviting attendees from Europe and Japan.

Upon discovery, Rett syndrome was once thought to be caused by degeneration of the central nervous system. However, it is now known that the disorder is a dysfunction of the aminergic neurons of the brainstem, starting during early infancy. Parents reported their infants were not quite right cognitively or praised the infant as a well-behaved babe, not crying and sleeping most of the day. This is because the poor function of the noradrenergic and serotonergic neurons residing in the brainstem.

It wasnít until 1986 that Dr. Hagberg and Dr. Witt-Engerstrom developed the classification of clinical staging of Rett syndrome. They proposed four age related stages that was later revised in 2001 based on pre regression, regression, and post regression. Regression refers to the period of time when an infant displays behaviors characteristic of autistic children consisting of delay in motor skills and speech. Following into early childhood there is a deceleration of head growth, muscle hypertonus starting in the legs, and shows signs of vulgus (6).





History Of Rett


Neuro Development


Progression of the Symptoms


The DMSís Definition


The Difference Between Rett and CDD


The Genetic Aspect


One Hopeful Treatment