PROGNOSIS FOR MENTAL FUNCTION IN APERTS-SYNDROME

The factors involved in the mental development of patients with Apert' s syndrome were studied by the authors, focusing on the age of the pat ient at operation, associated brain malformations, and the quality of the family environment. Overall, 32% of patients with significant foll ow-up review had an intelligence quotient (IQ) greater than 70. Age at operation appeared to be the main factor associated with changes in m ental development: final IQ was greater than 70 in 50% of patients ope rated on before 1 year of age versus only 7.1% in patients operated on later in life (p = 0.01). Malformations of the corpus callosum and si ze of the ventricles played no role in the final IQ, whereas anomalies of the septum pellucidum had a significant effect, with the proportio n of patients with an IQ over 70 increasing more than twofold in patie nts with a normal septum compared with patients with septal anomalies (p < 0.04). Quality of the family environment was the third factor inv olved in intellectual achievement: only 12.5% of institutionalized chi ldren reached a normal IQ level compared to 39.3% of children from a n ormal family background.