Elisabeth M. Dykens

Intellectual, academic, and adaptive functioning of Tourette syndrome children with and without attention deficit disorder

The intellectual, academic, and adaptive strengths and weaknesses of 30, medication-free children (M=10.5 years) with Tourette syndrome (TS) were assessed with a battery of standardized psychoeducational measures and the Vineland Adaptive Behavior Scales. Results indicated significant relative weaknesses in mental and written arithmetic, and relative strengths in reading achievement and abstact, logical thinking. Socialization skills emerged as a signifiant weakness in adaptive functioning. Comparisons between TS children with attention deficit disorder with hyperativity (ADD-H) (n=19) and without ADD-H(n=11) pointed to similar profiles of strength and weakness in both groups in all areas assessed, but significantly lower performance IQs in TS subjects with ADD-H. These findings are discussed in relation to future research with TS children.

Developmental implications of changing trajectories of IQ in males with fragile X syndrome

This study examined the trajectories of cognitive development in boys under the age of 21 years with fragile X syndrome. By combining information from three centers, data from 66 boys were analyzed; only children who had been tested two or more times with the same psychometric instrument at one or more year intervals were included in this study. Results demonstrated that males with fragile X syndrome show a decline in IQ scores, with the most marked declines seen during the early pubertal period. All 22 children retested during the 11- to 15-year period showed IQ declines, suggesting a slowing of development associated with the onset of puberty. Before age 10 years, males with higher (as opposed to lower) pretest IQs were more likely to decline at subsequent testings. A single etiological factor may not be sufficient to account for the observed findings, as both changes in neurobiological- and task-related factors seem implicated in the slowing intellectual development of this population.

Thought Disorder in High-Functioning Autistic Adults.

Examined thought disorder in a sample (n = 11) of high- functioning autistic young adults and older adolescents (mean IQ = 83) utilizing objective ratings from the Thought, Language and Communication Disorder Scale (TLC Scale) and projective data from the Rorschach inkblots. Results from the TLC Scale pointed to negative features of thought disorder in this sample (e.g., Poverty of Speech). Rorschach protocols revealed poor reality testing and perceptual distortions in every autistic subject, and also identified several areas of cognitive slippage (e.g., Incongruous Combinations, Fabulized Combinations, Deviant Responses, Inappropriate Logic). Comparing TLC Scale and Rorschach results to schizophrenic reference groups, autistic subjects demonstrated significantly more Poverty of Speech and less Illogically on the TLC Scale, and on the Rorschach they evidenced features of thought disorder that are encountered also in schizophrenia. Results are discussed in relation to the measures employed, and to areas of similarity and difference between autism and schizophrenia.

Trajectory of adaptive behavior in males with fragile X syndrome

Adaptive behavior in males with fragile X syndrome was longitudinally examined in 17 subjects, ages 1 to 17. Subjects received adaptive behavior evaluations on two occasions within one of three age periods. All domains of the Vineland Adaptive Behavior Scales increased from youngest to oldest age groups, yet older subjects (ages 10 to 17) shoed significant declines in their adaptive behavior scores from first to second testing. A relative strength in Daily Living Skills and weakness in Socialization emerged only among older subjects. There was a significant relationship between adaptive behavior and mental age scores in all subjects. Discussion emphasized the parallels between declines in IQ and adaptive behavior as well as the need for further research on adaptive skills in young adults with fragile X syndrome.

Genetics of Childhood Disorders: XV. Prader-Willi Syndrome: Genes, Brain, and Behavior

Prader-Wdli syndrome (PWS) is a developmental disorder characterized by mental retardation or learning disability, infantile hypotonia and poor suck reflex, growth retardation, delayed sexual development, and the childhood onset of pronounced hyperphagia Food-related difficulties are the most striking and widely recognized sequelae of this syndrome. Without appropriate dietary and behavioral intervention, almost everyone with PWS will become dangerously obese. However, in addition to these well-known problems with food, those with PWS also suffer from a range of psychiatric and behavioral difficulties, including high rates of depression, obsessions, and compulsions. PWS is a relatively rare disorder, with an incidence of approximately 1 in 10,OOO to 15,000 live births. Nonetheless, it has figured prominently in the recent history of genetics. This is due in large part to its surprising relationship with Angelman syndrome (AS) and the resulting identification of genomic imprinting in humans. Moreover, as research into the various aspects of this disorder has progressed, it has become increasingly evident that PWS may provide neuroscientists a valuable window into the complex interplay of genes, brain, and behavior. At first glance, the genetics of PWS appear to be rather straightforward. For most affected individuals, a sporadic (as

Trajectories and profiles of adaptive behavior in males with fragile X syndrome : Multicenter studies

We conducted two multicenter studies on adaptive trajectories and profiles in males with fragile X syndrome. Study 1 longitudinally assessed 29 males ages 1–20 years using ageequivalent scores from the Vineland Adaptive Behavior Scales. Fragile X boys ages 1–10 years showed significant gains in adaptive skills from first to second testing; males ages 11–20 years were stable in their adaptive development. Study 2 cross-sectionally examined 132 males ages 1–20 years. Significant age-related gains were found in boys ages 1–10, particularly in preschool children. Subjects ages 11–20 showed increased variability and nonsignificant relations between age and adaptive skills. Preliminary findings from 26 young adults with fragile X syndrome ages 21–40 years showed stable age-equivalent adaptive scores during these years. Relative strengths in daily living skills and weaknesses in communication were only evident among older subjects. Significant relations were found between adaptive behavior standard scores and IQ; these two scores also showed age-related declines that likely parallel one another. Findings are related to adaptive features in other genetic syndromes, and to directions for future adaptive behavior research.

Cognitive, Behavioral, and Adaptive Functioning in Fragile X and Non-Fragile X Retarded Men

The cognitive, behavioral, and adaptive functioning of 12 men with fragile X syndrome (aged 23 to 62 years) was systematically assessed and compared to two matched groups of retarded men without fragile X syndrome residing at the same institution. The fragile X group was largely indistinguishable from the camparison groups on the cognitive, behavioral, and adaptive measures. Fragile X patients were, however, significantly more likely to have achieved levels of adaptive functioning commensurate with their intellectual abilities. Fragile X subjects who had similarly affected siblings emerged as significantly higher-functioning in all areas than Fragile X subjects who did not have affected siblings. These findings are discussed with respect to future research.

Changing patterns of intellectual strengths and weaknesses in males with fragile X syndrome

Examined the changing profiles of intelligence in males with fragile X syndrome as these individuals increased in chronological age. Using a psychometric instrument designed to measure styles of information processing, 21 males aged 4 to 27 years were examined cross-sectionally in sequential processing, simultaneous processing, and achievement. The age of the subject was associated with age-equivalent levels of both simultaneous processing and achievement, but fragile X males did not show higher levels of sequential processing with increasing chronological age. Compared to younger fragile X males, the older subjects were more delayed in sequential processing skills relative to their abilities in other areas. A smaller longitudinal study confirmed the presence of a plateau in sequential processing among those subjects tested two times after the age of 10 years. Implications are discussed for diagnosis, intervention, and the matching of subject groups in mental retardation research.

The Draw-a-Person Task in Persons with Mental Retardation: What Does It Measure?.

As a widely used, easy-to-administer, and nonthreatening task, the Draw-a-Person (DAP) holds particular promise as a nonverbal index of intelligence in persons with mental retardation. The DAPs of 108 adolescents and adults with mental retardation were reliably scored using Naglieris cognitive and emotional disturbance scoring systems. Contrary to expectations, visual-motor skills emerged as the best predictor of DAP cognitive scores. Intelligence was correlated with DAP scores, but had considerably less predictive value than visual-motor skills. DAP emotional indicator scores were only modestly associated with social adaptation; even less support was found linking DAP emotional indicators to psychopathology. Findings point to considerable caution in using the DAP as an index of intelligence or as a screen for adjustment problems or specific psychopathology. Although related to intelligence, this popular task seems predominantly to measure visual-motor development in adults with mental retardation.

Maternal Emotional Reactions to Young Children with Different Types of Handicaps

Emotional reactions were examined cross-sectionally over the 1-to 6-year period in mothers of children with Down syndrome (N = 25) and with predominantly motor impairments (N = 20). Mothers rated their degree of concern about 16 early milestones and four events, and were also interviewed with the Vineland Adaptive Behavior Scales. Although milestones occurring during the first year caused higher levels of maternal concern than did later-occurring ones, concern was also evident for some later milestones. Mothers of Down syndrome and of motor-impaired children were equally concerned about motor milestones, but mothers in the Down syndrome group were more concerned about developments in communication. The two groups modulated their maternal concern based on different aspects of the childs communication skills. The timing, specificity, and sensitivity of maternal reactions identified in this study are discussed in relation to the unidirectional, time-bound model of “maternal mourning.” J Dev Behav Pediatr 13:118–123, 1992.