In this report we present the results of a study of the intellectual functioning and cognitive profile of 26 Prader‐Willi syndrome (PWS) patients. The mean IQ score was 62.3 (range 39–96). In 13 patients a significant difference between verbal and performance IQ was found. In 10 of them the performance IQ was higher than the verbal. The results of subtest analysis indicate that cognitive strengths are more visible than cognitive weaknesses. Highest scores were noted especially in the performance scale, i.e. Block Design (9 children) and Coding or Mazes (5 children). Analysis of all available data indicates that PWS patients score better on visual motor discrimination skills than on auditory verbal processing skills. These results are promising for intervention programs and education stiategies.

In this study we present data on the adaptive behavior profile and on the development of adaptive functioning in 39 fragile X [fra(X)] males, age 4-26 years. Social adaptability is relatively well developed as compared to cognitive level and especially self-help skills continue to grow with age despite a stagnation in intellectual growth.

ABSTRACT. In this study clinical and psychological findings arc presented in live 49.XXXXY patients. Their degree of mental retardation varied greatly, i.e. from moderalely to profoundly retarded. A decline in intelligence performance with age was obserived in one boy. Language developmenl was severely retarded with a remarkable discrepancy between language expression and comprehension. Emotional disturbances with low frustration level, timidity and shyness were noted in all live and their level of adaptive functioning was much higher than the cognitive level.

SUMMARY A longitudinal study of IQ and cognitive profile in 18 fra(X) positive boys is reported. At the time of diagnosis, four of the boys were mildly retarded, seven were moderately retarded, and five were severely mentally retarded. Intelligence was borderline in one child and normal in another. A decline in intellectual performance with age in the fra(X) syndrome indicated in previous studies was not confirmed and we review the reported data on this subject.In the various studies of the psychological profile of the fra(X) syndrome consideration has been given to the intellectual functioning of these males. In the initial studies a global estimation of their intellectual performance was given with or without further data on exact IQ measurements. In the majority of these reports, however, precise data on the type of IQ tests were not given. evaluated on the basis of these traditional intelligence tests and in only a minority was consideration given to the broader spectrum of cognitive function. In the fragile X syndrome few data are available on the development of cognitive function and performance in relation to age. In most reports the authors discussed the results of cross sectional studies7 15 17 22 24 25 and in the majority a decline in IQ in relation to age was reported.The aim of the present study was to determine the fluctuation of IQ with age and to elaborate the different cognitive functions of fra(X) boys. Methods and resultsIn this study intellectual function and cognitive profile of 18 fra(X) positive males* was analysed.One of the patients is an adult.

In this study we investigated the development of adaptive behavior of 8 fragile X [fra(X)] males with special attention to social competence and compared the results with a control group of 8 fra(X) negative males matched for age, level of adaptive functioning, and period of institutionalization.