An experimental approach to detecting dementia in Down syndrome: a paradigm for Alzheimer's disease

Measures developed from animal models of aging may detect dementia of the Alzheimer's type in a population at-risk for Alzheimer's disease (AD). Although, by middle age, individuals with Down syndrome (DS) show an extraordinarily high prevalence of AD-type pathology, their severe idiopathic cognitive deficits tend to confound the clinical diagnosis of AD. The current study was designed to improve detection of AD in DS by using measures of learning and memory derived from animal models of aging. Adults with DS (N=34) were assessed and reassessed (n=19) approximately one year later using stimulus-response (S-R) test methods derived from experimental literature, as well as standardized informant-based tests. Results demonstrated high validity and reliability of select tests. The implication of early symptom detection in a population at-risk for AD-type dementia was discussed in terms of potential brain regions of interest.     

Comorbidity in allergic asthma and allergic rhinitis: functional somatic syndromes

Based on the concept of central sensitisation, the present study tested the hypothesis of comorbidity in allergic asthma and allergic rhinitis with diagnoses of functional somatic syndromes (FSSs), including fibromyalgia, irritable bowel syndrome and migraine. Data were used from the population-based Västerbotten Environmental Health Study (n = 3406). The participants consisted of 164 individuals with allergic asthma and 298 individuals with allergic rhinitis as well as 2876 individuals without allergic or non-allergic asthma, allergic rhinitis or atopic dermatitis. Diagnoses were based on self-reports of having been diagnosed by a physician. Odds ratios (ORs) were calculated from binary logistic regression analysis, both crude and adjusted for age and education. The adjusted ORs (1.87–4.00) for all FSSs differed significantly from unity for both allergic asthma and rhinitis. The results provide support for the hypothesis of comorbidity in allergic asthma and rhinitis with FSSs. Since central sensitisation is likely to underlie FSSs, the present findings raises the question as to whether central sensitisation may also be involved in allergic asthma and rhinitis.     

An evaluation of the effectiveness of exposure and response prevention on repetitive behaviors associated with Tourette's syndrome

Exposure and response prevention (ERP) was evaluated as treatment for three repetitive behaviors in an 11-year-old boy using a multiple baseline across behaviors design. The repetitive behaviors and associated self-reported distress were eliminated. At 3-month follow-up, the frequency for two of the three behaviors returned to baseline levels. This study demonstrates that ERP may be a useful treatment for repetitive behaviors, although booster sessions may be needed to maintain the treatment effects.     

The Cultural Education of Academic Evaluators: Lessons from a University-Hmong Community Partnership

This article explores some of the lessons a university-based evaluation team learned while attempting to hire, retain, and work with staff members from a local Hmong community. These staff members were hired to assist a federally-funded community collaborative with implementing and evaluating a family strengthening program for Hmong families with adolescents. Over the course of two years, a succession of individuals were hired and resigned from a key staff position. These resignations occurred at critical points in the project, undermining a critical component of the program's evaluation. Through interviews, observations, self-reflection, and feedback from colleagues and reviewers, a better understanding of the reasons for these difficulties began to emerge. The challenges examined in this article help to illustrate some of the complexities faced by academics when conducting a community-based project with an underserved racial ethnic population. The article concludes by discussing some of the lessons learned and what they might suggest for others doing similar types of work.     

What makes counting count? Verbal and visuo-spatial contributions to typical and atypical number development

Williams Syndrome (WS) is marked by a relative strength in verbal cognition coupled with a serious impairment in non-verbal cognition. A strong deficit in numerical cognition has been anecdotally reported in this disorder; however, its nature has not been systematically investigated. Here, we tested 14 children with WS (mean age=7 years 2 months), 14 typically developing controls individually matched on visuo-spatial ability (mean age=3 years 5 months) as well as a larger group of typically developing controls (mean age=3 years 4 months) on two tasks to assess their understanding that counting determines the exact quantity of sets (cardinality principle). The understanding of the cardinality principle in children with WS is extremely delayed and only at the level predicted by their visuo-spatial MA. In this clinical group, only language accounted for a significant amount of the variance in cardinality understanding, whereas in the normal comparison group only visuo-spatial competence predicted the variance. The present findings suggest that visuo-spatial ability plays a greater role than language ability in the actual development of cardinality understanding in typically developing children, whereas the opposite obtains for the clinical group.     

Treatment of vocal tics in children with Tourette syndrome: investigating the efficacy of habit reversal

Habit reversal was used to treat vocal tics in 5 children with Tourette syndrome. Vocal tics were reduced in 4 of the 5 children, the untreated motor tics did not increase, and treatment was acceptable to the children's parents.     

What Predicts the Use of Genetic Counseling Services After the Birth of a Child with Down Syndrome?

In the state of Victoria, Australia, a government funded genetic counseling service exists to meet the needs of families. An audit showed that many families do not use this service after the birth of a child with a genetic problem. To investigate this we surveyed families of children born with Down syndrome over 2 years in Victoria. Questionnaires were completed by 74 mothers, of whom only 18 had received genetic counseling between the birth and the time of the study (mean 3.5 years). Of those not receiving genetic counseling, 71% said they were not offered or had not heard of it. Mothers who had genetic counseling were younger than those who had not, and were more likely to have attended University. Those who had genetic counseling indicated less satisfaction with care at the diagnosis and were more likely to perceive their child as unwell at birth than those who were aware of genetic counseling but did not have it. Of those who did not have genetic counseling, over half were unclear about what it is, although 74% agreed with the statement genetic counseling is most useful when planning to have another child. Of those who had heard of genetic counseling, 73% said they were not sure how it could help. Many families with children with Down syndrome are not aware of the existence or functions of genetic counseling. With greater awareness, some may still choose not to have genetic counseling, but others enunciated needs that could be met by this service if it were offered to them.     

Neuropsychological Performance in Gulf War Era Veterans: Motivational Factors and Effort

Assuming that the underlying etiology of unexplained health-related symptoms in returning Gulf War (GW) veterans is multifactorial, the possible role of feigning or exaggeration of symptoms is worth consideration as a contributing factor. The present study assessed the relationship between motivation to perform well during neuropsychological assessment and objective neuropsychological test performance. Motivation was measured as the score on a visual memory task (Test of Memory Malingering, TOMM) of low difficulty. Participants included 77 veterans from the cohorts of GW- (n = 58) and Germany-deployed (n = 19) GW-era veterans described in two other papers in this issue who were administered the TOMM. Most veterans earned perfect or near-perfect scores on the TOMM (48–50/50). Scores 47 were associated with lower scores on neuropsychological tasks assessing attention, executive functions, and memory. Variability in test performance within and between tasks measuring similar functions was also found in participants with lower TOMM scores.