[Clinical study of four cases of Rett Syndrome]

Rett Syndrome was first described in 1966 by Andreas Rett. The disorder is characterized by a progressive loss of cognitive and motor skills as well as development o stereotyped hand movements, occurring after an apparently normal development. Authors present three typical cases, and, another one atypical, being all of them female. This study takes into account ten different areas about chronology, age and reasons in the first consultation, some milestones of psychomotor development, the diagnostic criteria--according to Rett Syndrome Diagnostic Criteria Work Group (R.S.D.C.W.G.), they consists of necessary, supportive and exclusion criteria-, some signs and symptoms that authors consider frequently associated with Rett syndrome, some diagnostic tests with neurophysiologic technics--E.E.G.--, as well as neuroimagins technics--C.T. and M.R.I.--; routine laboratory studies, development scales and something else, the DAS-SV scale which is usually used to study different handicaps and therapeutic interventions such as: psychosocial, clinical, pharmacological (valproic acid, carbamacepine, clorpromacine, etc.) and institutional. Three tables and 72 bibliographic notes are included.