Metachromatic Leukodystrophy and Nonverbal Learning Disability: Neuropsychological and Neuroradiological Findings in Heterozygous Carriers

Metachromatic leukodystrophy (MLD) is an autosomal recessive neurodegenerative disorder due to deficiency of the enzyme arylsulfatase A that leads to progressive, diffuse demyelination. The syndrome of nonverbal learning disability has been attributed to white matter abnormality and has been reported in children with this disorder and in some healthy family member carriers of gene. We examined the neuropsychologic profiles and MRIs of eight members of the family of a 7-year-old girl with this disease, all of whom were heterozygous carriers of the mutation and five of whom were also carriers of the MLD pseudodeficiency gene. All had low normal levels of arylsulfatase A, and seven of the eight had average or better profiles across all assessed neuropsychological domains. The patient's younger sister had a profile with features of the syndrome of nonverbal learning disability despite a normal MRI, whereas two members with minor white matter findings did not. This family does not provide evidence for the syndrome of nonverbal learning disability in heterozygous carriers of the gene for MLD, even when associated with the MLD pseudodeficiency gene.     

16PF in screening for appropriateness of mentors

The utility of the Sixteen Personality Factor Questionnaire, Fifth Edition (16PF) as an indicator of mentor effectiveness was examined. A random sample of the 16PF scores of 74 mentors was drawn from a population of 837 mentors from Big Brothers Big Sisters. Caseworkers rated mentor's effectiveness using a rubric developed for this purpose. The rubric showed good interrater agreement. Caseworkers' ratings of mentor's effectiveness was used to rate mentors systematically as appropriate or inappropriate. The 16PF scores of mentors were compared at an alpha level of .05 for appropriate and inappropriate groups using independent t tests and multivariate analyses of variance, which reflected significant differences between male and female mentors on Factors E and Q3. Significant differences were also found between "appropriate" and "inappropriate" mentors on Factors L and Q4. These differences reflected only moderate effect sizes and lacked practical significance or meaning. The results suggest that, while the 16PF discriminates statistically between "appropriate" and "inappropriate" mentors, in terms of practical significance, the questionnaire is not particularly useful as an initial screening tool.     

Applications of social cognitive theory to the career development of women of color

This article provides a review of the literature on the educational and career development of women of color within the context of social cognitive career theory (Lent, et al, 1994 and Lent, et al, 1996). Specifically, possible gender, ethnic, racial, and cultural influences on career self-efficacy and outcome expectations are explored to delinate the potential mechanisms whereby ethnicity, culture, and ethnic and racial identity development, in interaction with gender, affect career choice and adjustment. Suggestions for future research on social cognitive career theory and the career development of women of color are presented.     

Longitudinal Assessment of Pain, Coping, and Daily Functioning in Children with Sickle Cell Disease Receiving Pain Management Skills Training

Objective. To conduct intensive pain management skills training (IST) in children with sickle cell disease (SCD) and their parents and to comprehensively evaluate pain, coping, and daily functioning in children pre, immediately post, and 3 months following treatment. Methods. Three children who received IST in nonpharmacological and pharmacological pain management strategies completed a Coping Strategies Questionnaire (CSQ) at pre, post, and follow-up assessments, and daily pain and activity diaries for 18 weeks, spanning from 1 week pretreatment to 11 weeks posttreatment. Results. From pre- to posttreatment, 1 child receiving IST indicated increased report of active coping attempts and all 3 children indicated decreased report of negative thinking on the CSQ. Participants in IST used coping skills on 90% of days with pain and reported the skills to be moderately helpful on the daily diaries. For daily activities such as eating dinner, playing with friends, and hours slept, children participated similarly on days with pain and days without pain during the posttreatment period. Given the small number of participants in this study, individual cases are discussed to highlight similarities and differences in how participants responded to the treatment and during the 3-month follow-up period. Conclusions. In this pilot study, each participant showed improvement in coping and daily functioning after completing the IST program. Individual differences in response to treatment indicate the need for more targeted intervention programs that incorporate pharmacological and nonpharmacological components. The results of this study highlight both the promise and the complications of conducting comprehensive pain intervention and functional outcome studies in children with SCD.     

Metachromatic leukodystrophy and nonverbal learning disability: neuropsychological and neuroradiological findings in heterozygous carriers.

Metachromatic leukodystrophy (MLD) is an autosomal recessive neurodegenerative disorder due to deficiency of the enzyme arylsulfatase A that leads to progressive, diffuse demyelination. The syndrome of nonverbal learning disability has been attributed to white matter abnormality and has been reported in children with this disorder and in some healthy family member carriers of gene. We examined the neuropsychologic profiles and MRIs of eight members of the family of a 7-year-old girl with this disease, all of whom were heterozygous carriers of the mutation and five of whom were also carriers of the MLD pseudodeficiency gene. All had low normal levels of arylsulfatase A, and seven of the eight had average or better profiles across all assessed neuropsychological domains. The patient's younger sister had a profile with features of the syndrome of nonverbal learning disability despite a normal MRI, whereas two members with minor white matter findings did not. This family does not provide evidence for the syndrome of nonverbal learning disability in heterozygous carriers of the gene for MLD, even when associated with the MLD pseudodeficiency gene.     

Applications of social cognitive theory to the career development of women of color

This article provides a review of the literature on the educational and career development of women of color within the context of social cognitive career theory (Lent, et al, 1994 and Lent, et al, 1996). Specifically, possible gender, ethnic, racial, and cultural influences on career self-efficacy and outcome expectations are explored to delinate the potential mechanisms whereby ethnicity, culture, and ethnic and racial identity development, in interaction with gender, affect career choice and adjustment. Suggestions for future research on social cognitive career theory and the career development of women of color are presented.