Juvenile onset Huntington's disease--clinical and research perspectives

Huntington's disease (HD) is an inherited neurodegenerative disorder. The mutation which causes the disease is an expansion in the number of repetitions of three nucleotides, C, A, and G in exon 1 of the huntingtin gene. The gene normally has 15 to 30 repeats and an expansion to 40 or more is associated with HD. HD usually has a mid-life onset, but a juvenile form, defined by onset of symptoms before the age of 21 years, is present in about 7% of HD cases. Juvenile HD is characterized by (1) transmission from an HD affected father, (2) an unusually large repeat size, usually of 60 or more units, and (3) unique clinical features, including rigidity and seizure disorder. Although juvenile onset is associated with a more severe neuropathological involvement, the neuropathological characteristics of juvenile HD are similar to those seen in the adult form in that the striatum bears the brunt of the illness. Clumps of protein, termed inclusion bodies, which stain positive for huntingtin and ubiquitin, are found primarily in the nucleus but also in the cytoplasm and axons in HD neurons. Research suggests that these inclusion bodies sequester a deleterious protein fragment and prolong cell life during the degenerative process of the disease.     

Confessions from the Classroom Teaching with Augustinian Eyes

Through an analysis of Augustine's Confessions, this essay aims to identify the sources, tenets, and implications of the theological anthropology that grounds the author's pedagogy. The author describes classroom dynamics and teaching strategies in terms of the concepts of creation, sin, and redemption found in the Confessions. In relation to Augustine's doctrine of creation, the author argues that a theological anthropology that posits an ineradicable relationship of the human person to God justifies optimism about student response to the study of theology. It also supports a sacramental understanding of the effectiveness of the teacher. In relation to Augustine's theology of sin, the author reflects on the effects of pride on both teacher and student. The section on redemption acknowledges that although the teacher cannot eradicate sin in the classroom, he or she can counter such effects through the responsible and sensitive exercise of authority. Throughout the essay, the virtues of humility and gratitude in the classroom are highlighted, and concrete pedagogical issues are examined in a theological light.     

Familiarity and pronounceability of nouns and names

Ratings of familiarity and pronounceability were obtained from a random sample of 199 surnames (selected from over 80,000 entries in the Purdue University phone book) and 199 nouns (from the Ku?era-Francis, 1967, word database). The distributions of ratings for nouns versus names are substantially different: Nouns were rated as more familiar and easier to pronounce than surnames. Frequency and familiarity were more closely related in the proper name pool than the word pool, although both correlations were modest. Ratings of familiarity and pronounceability were highly related for both groups. A production experiment showed that rated pronounceability was highly related to the time taken to produce a name. These data confirm the common belief that there are differences in the statistical and distributional properties of words as compared to proper names. The value of using frequency and the ratings of familiarity and pronounceability for predicting variations in actual pronunciations of words and names are discussed.     

Mother Knows Best? Mother and Child Report of Behavioral Difficulties of Children of HIV-Infected Mothers

Children of HIV-infected mothers have more psychosocial adjustment difficulties than do those of noninfected mothers. In this study, child psychosocial adjustment in children of HIV-infected women is examined across the three stages of HIV-infected: asymptomatic, symptomatic and AIDS. Participants were 99 HIV-infected women and 148 noninfected women. Children were not identified as being HIV infected. Mother and child reports of behavioral difficulties are compared, and competing hypotheses about mother report of child behavior difficulties are tested. Results indicate an ascending linear trend for child report of internalizing and externalizing difficulties from the noninfected stage through increasingly severe stages of HIV-infection. According to mother report, child externalizing and internalizing difficulties are nonlinear, as mothers report an increase through the infected symptomatic stage, then a decrease in the AIDS stage. Implications for assessment, prevention and intervention in families with maternal HIV infection are discussed.     

Counseling Women in Midlife: An Integrative Approach to Menopause

Menopause is a normative life transition that is experienced by virtually all women, yet remains poorly understood. The dominance of the biomedical model results in a unidimensional definition of menopause as a biological event, ignoring the influence of cultural and psychosocial factors. Counselors who use an integrative approach can not only provide accurate biomedical information but also conceptualize menopause as a normative midlife transition. The purpose of this article is to provide a foundation in biomedical issues and to consider how these independently, and in interaction with cultural and psychosocial factors, influence the experience of menopause.     

Toward a Developmental Diagnostic and Statistical Manual: The Vitality of a Contextual Framework

Counselors of all types need an understanding of and an ability to work with issues described by the Diagnostic and Statistical Manual of Mental Disorders, fourth edition (DSM-IV; American Psychiatric Association, 1994). The traditional approach to the DSM-IV tends to locate the problem in the client, whereas a developmental approach focuses on the client in social and historical context. Specific recommendations for organizing a comprehensive treatment plan are presented. The authors endorse an educational and developmental role for counselors.     

Potential Bone-Marrow Donors and Their Spouses: The Effects of Volunteerism on Distress Levels1

The current study sought to provide a better understanding of the reactions of bone-marrow volunteers to the possibility of donating by examining these volunteers in the context of their closest relationships. Using Tesser's (1988) self-evaluation maintenance (SEM) model as a guide, we examined the influence of spouses who are also volunteers on each member of the couple's distress levels and on the marrow volunteer's ambivalence about donating. We also examined the independent effects of known risk factors for donation-related distress and the combined effects of spouse volunteer status and these risk factors on distress levels. Questionnaire data were collected from 278 couples selected from 39 bone-marrow donor centers. Results demonstrated that when spouses of potential donors were engaged in any type of volunteer activity, members of the couple felt greater anxiety than when spouses were not volunteers. Additionally, other variables were found to have a conjoint effect with spouse volunteer status on volunteer and spouse anxiety levels.