Rett Syndrome: An Analysis Using Sartwell's Incubation Period Model

Abstract

Evidence suggests that Rett syndrome is a genetic disorder; however, an etiologic genetic model has yet to be identified. The purpose of this study was to apply a statistical model, Sartwell's incubation period model, to estimate the incubation period of Rett syndrome and postulate further on the etiology of Rett syndrome. Sartwell's model was used to test the hypothesis that the age of onset of developmental regression distributions approximate a logarithmic normal model and thus the etiologic factors of Rett syndrome occur in utero or prior to conception. Data from three case-series of Rett syndrome from different geographic regions were used for the analyses. Curves resulting from the analyses demonstrated a good approximation to the logarithmic normal distribution, indicating a good fit of the data to Sartwell's model. In conclusion, the incubation period of Rett syndrome fits the logarithmic normal model, which is consistent with the theory that a major causal factor for Rett syndrome most likely occurs in utero or prior to conception, eg, a defective gene. (J Child Neurol 1997;12:361-364).