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Early stages of the Rett syndrome and infantile neuronal ceroid lipofuscinosis--a difficult differential diagnosis.

The Rett syndrome (RS) and infantile neuronal ceroid lipofuscinosis (INCL) are known clinically to present with quite similar manifestations in the very first stages of disease. Ten girls, born 1982-85, from the Swedish series of RS, were compared with 6 children with biopsy-confirmed INCL, deriving from 4 families, as to neurodevelopmental, neurological, ophthalmological, neurophysiological, and CT data. The rapid regression at 1-2 years of age with loss of acquired fine motor skill, learned words, and communication were found to be inseparable between the diseases, as were the successively developing hand and finger stereotypies. Early clues for differential diagnosis were the appearance of transient drop spells, loss of head control, and irregular myoclonias in INCL, flattening of the EEG, early cortical atrophy indicated at CT, and particularly abnormal ERGs. For accurate diagnosis of INCL a biopsy with characteristic EM findings of "snowball" aggregates is a necessity. After 2 3/4-3 years of age, a clinical differentiation was possible in all cases, with visual failure and rapidly decreasing motor ability, head control deterioration, hyperexcitability, and trunk-limb extension tonus being characteristic for INCL.

Child, Preschool↗

Media and civic socialization of youth.

Research on the development of citizenship has been reinvigorated by considering adolescents as participants actively engaged in, and interacting with, family, peers, teachers, and the media. This contrasts with earlier top-down transmission models that saw adolescents as passive recipients of information from parents and teachers. Active citizenship is now seen as a largely indirect result of contextualized knowledge and cognitive skills learned from news media use, interpersonal communication, and active participation in school and community volunteer activities. The processes of active citizenship learning are an important part of the moral development of adolescents and young adults.

Adolescent↗

Self-management for the psychiatric patient at home.

Psychiatric patients in home care require support and treatment, but they also need to learn skills and self-management that will assist them after home care ends. Mental illness can be compared with any long-term illness, such as diabeteS or arthritis, in that patients should be prepared to cope with their illness through self-management. Mental illness should be considered no differently because patients also experience fluctuation in symptoms as a result of stress and the changing nature of their illnesses and will not always require intensive treatment.

Aftercare↗

Fostering evidence-based care in clinical teaching.

Understanding scientific research evidence and applying it to clinical practice is a focus in today's health care arena. Fostering evidence-based care (EBC) in clinical teaching is an effective way to help students learn to think critically, search for, evaluate, and incorporate the best research evidence into their clinical practice. For example, helping a student plan care for a woman creates a "teaching moment" for the application of scientific evidence to clinical practice. Essentially, EBC is a learned skill for both the student and the clinical teacher. It requires intellectual curiosity and a willingness to explore beyond "the way it has always been done." This article presents a review of the goals of clinical teaching, strategies to foster EBC, and tools to assess the readiness of the teacher, the student, and the clinical setting for EBC. By aligning the goals of clinical teaching with evidence-based strategies, both the educator and student strive together to provide midwifery care that is supported by available research.

Clinical Competence↗

Ecological implications of limb apraxia: evidence from mealtime behavior.

Humans learn skilled acts in order to effectively interact with their environment. A loss of the ability to perform skilled acts is termed apraxia. Apraxia has been thought to be of theoretical interest, but the ecological implications of apraxia are controversial and have not been fully studied. We examined ten patients with unilateral left hemisphere cerebral infarctions (eight of whom were apraxic) and compared their mealtime eating behavior to a group of neurologically normal, age-matched controls. The stroke patients were less efficient in completing the meal. They made more action errors and were less organized in the sequencing of mealtime activities. Because the patients made more errors while using tools than when performing nontool actions, their deficit could not be accounted for by an elemental motor deficit. A positive relationship was found between mealtime action errors and the severity of apraxia. These findings suggest that limb apraxia may adversely influence activities of daily living.

Activities of Daily Living↗

Improving access for Latino immigrants: evaluation of language training adapted to the needs of health professionals.

The number of Latinos in North Carolina grew by almost 400% between 1990 and 2000. The rapid change in demographics in this state and other southeastern states has caught healthcare providers unprepared. Lack of ability to communicate with Latino patients may result in errors in diagnosis or reduced compliance with recommended treatments. The Culturally and Linguistically Appropriate Services (CLAS) Standards, published in 2001, mandate culturally and linguistically appropriate services for persons with limited English proficiency. This paper describes an innovative strategy to promote Spanish and culture-learning skills of healthcare providers and presents results of the evaluation conducted to determine its impact on access to quality care. The evaluation used a 360 degrees case study design, at 1-year follow-up. Use of Spanish language health-related materials is key to the training's success. The authors make recommendations for replication of the integrated language and culture-training model in other new settlement areas, especially those in the southeast of the United States.

Communication Barriers↗

Cognitive-behavioral stress management intervention buffers distress responses and immunologic changes following notification of HIV-1 seropositivity.

Forty-seven asymptomatic, healthy gay men were randomly assigned to a cognitive-behavioral stress management (CBSM) condition or an assessment-only control group 5 weeks before being notified of their HIV-1 antibody status. Seventy-two hours before and 1 week after serostatus notification, blood samples and psychometric data were collected. Control subjects showed significant increases in depression, but only slight decrements in mitogen responsivity and lymphocyte cell counts pre- to postnotification of seropositivity. Seropositive CBSM Ss did not show significant pre-post changes in depression, but did reveal significant increases in helper-inducer (CD4) and natural killer (CD56) cell counts as well as a slight increment in proliferative responses to phytohemagglutinin (PHA). Individual difference analyses suggest that the psychological buffering and immunomodulating effects of the CBSM manipulation may be attributable, in part, to relaxation skills learned and practiced or to a general willingness to comply with the intervention guidelines.

AIDS Serodiagnosis↗

On the relationship between recall and recognition memory.

The relationship between recall and recognition has been a central topic for the study of memory. A test of alternative views about recall and recognition was arranged by studying amnesic patients. In amnesia, damage has occurred to a brain system important for declarative (conscious) memory, but skill learning, priming, and other forms of nonconscious memory are intact. Recall and recognition were found to be proportionately impaired in amnesic patients, and confidence ratings for the recognition judgments were commensurate with the level of impaired performance. The results are contrary to views that either recognition memory or associated confidence judgments are ordinarily supported significantly by nonconscious memory. The results favor the view that recall and recognition are related functions of declarative memory and equivalently dependent on the brain system damaged in amnesia.

Aged↗

Medial temporal lesions in monkeys impair memory on a variety of tasks sensitive to human amnesia.

Monkeys with conjoint bilateral lesions of the hippocampus and amygdala were impaired on four different tests of memory (delayed retention of object discriminations, concurrent discrimination, delayed response, and delayed nonmatching to sample). Because tests involving delays and distractions are known to be especially sensitive to human amnesia, in three of the tasks relatively long delay intervals between training and test trials were used, and in two tasks distraction was introduced during the delay intervals. The severity of the impairment increased with the length of the delay, and distraction markedly increased the memory impairment. For one task given on two occasions (delayed nonmatching to sample), the severity of the impairment was unchanged over a period of 1.5 years. Taken together with previous findings that skill learning is unimpaired in the same operated monkeys, the results of the present study strengthen the conclusion that monkeys with medial temporal lesions constitute an animal model of human amnesia. In addition, the four tasks used here appear to constitute a sensitive and appropriate battery that could be used in other studies of the neuroanatomy of memory functions in the monkey.

Amnesia↗

Age differences in implicit memory: fragmented object identification and category exemplar generation.

In a cross-sectional study of 164 participants aged 21 to 91, the authors examined age differences on two implicit tests, fragmented object identification (FOI) and category exemplar generation (CEG), and on tests of explicit memory, attention, and verbal fluency. FOI results revealed impaired perceptual skill learning in those over 60 and a decrease in perceptual priming across young, middle-aged, and older groups. CEG priming was impaired in those over 80. Regression analysis revealed explicit contamination of priming on both the FOI and CEG tests. Across the three implicit measures, age accounted for 4 to 13% of the variance when explicit memory was controlled. Semantic fluency predicted CEG priming, suggesting possible frontal lobe involvement on the test. Altogether, results indicate that age has a small but reliable influence on implicit memory.

Adult↗

Ability and task constraint determinants of complex task performance.

Previous research on basic information-processing tasks has suggested that there may be a dissociation between the underlying process determinants of task performance and associations with ability measures. This study investigates this dissociation in the context of a complex skill-learning task-an air traffic control simulation called TRACON. A battery of spatial, numerical, and perceptual speed ability tests were administered, along with extensive task practice. After practice, manipulations of task requirements and system consistency were introduced. Ability correlations with performance revealed a dissociation between some manipulations that have effects on performance means and the corresponding correlations with reference abilities. Implications for integrating experimental and differential approaches to explaining performance and possible avenues for improved selection measures are discussed.

Adolescent↗

Sleep apnoea related hypoxia is associated with cognitive disturbances in patients with tetraplegia.

Sleep disordered breathing is common in patients with tetraplegia. Nocturnal arterial hypoxemia and sleep fragmentation due to sleep apnoea may be associated with cognitive dysfunction. We therefore studied the influence of sleep disordered breathing on neuropsychological function in 37 representative tetraplegic patients (mean age 34 +/- 9.7 years). Thirty percent (11 of 37 patients) had clinically significant sleep disordered breathing, defined as apnoea plus hypopnoea index (AHI) greater than 15 per hour of sleep. Most apnoeas were obstructive in type. Seven patients (19%) desaturated to < 80% during the night. Neuropsychological variables were significantly correlated with measures of sleep hypoxia, but not with the AHI and the frequency of sleep arousals. The neuropsychological functions most affected by nocturnal desaturation were: verbal attention and concentration, immediate and short-term memory, cognitive flexibility, internal scanning and working memory. There appeared to be a weak association between the presence of severe sleep hypoxia and visual perception, attention and concentration but no association was found between sleep variables and depression scores. We concluded that sleep disordered breathing is common in patients with tetraplegia and may be accompanied with significant oxygen desaturation. The latter impairs daytime cognitive function in these patients, particularly attention, concentration, memory and learning skills. Cognitive disturbances resulting from sleep apnoea might adversely affect rehabilitation in patients with tetraplegia.

Adolescent↗

Identifiers of predominantly Spanish-speaking children with language impairment.

The purpose of this study was to identify a set of measures that would discriminate 31 predominantly Spanish-speaking children with normal language (NL children) from 31 children with language impairment (LI children). The LI children were identified as such by experienced, bilingual (Spanish/English), ASHA-certified, speech-language pathologists who were currently seeing the children in their caseloads. Children ranged in age from 5 to 7 years and were matched for age, gender, and school. Additionally, nonverbal cognitive measures assured that they did not differ significantly intellectually. Measures of vocabulary, novel bound-morpheme learning skills, and language form were randomly administered to all children. Further, parents responded to questions about their perceptions of their children's speech and language skills and family history of speech and language problems. A stepwise discriminant analysis indicated that 4 measures discriminated the groups of children with a sensitivity of 91.3% and a specificity of 100% (p < .0001): parental report of the child's speech and language skills, number of errors per T-unit, mean length per T-unit, and family history of speech and language problems. A second discriminant analysis indicated that the sensitivity and specificity could be maintained when only the first 2 measures were included. Confirmatory discriminant analyses of the 2- and 4-measure models indicated that the discriminant accuracy was stable on an independent sample.

Child↗

Acceptability and Feasibility of an Educational Intervention to Improve Researcher-Participant Interactions in a Neonatal Intensive Care Unit Clinical Trial: Research Team Feedback on the BRIEF Intervention.

OBJECTIVE: Interactions with families are essential to successful recruitment conversations that promote informed decision-making about clinical research enrollment. However, there is little evidence about how to implement communication-oriented recruitment training among pediatric clinical research teams. Our objective was to evaluate the feasibility and acceptability of Better Research Interactions for Every Family (BRIEF), a multipart educational intervention to improve relationship-based conversations about clinical trial enrollment with families in the neonatal setting. STUDY DESIGN: We piloted BRIEF in partnership with a neonatal clinical research team. Research team members completed surveys following the BRIEF intervention's online module and the BRIEF group training session. They completed self-assessments after consent discussions before and after the BRIEF intervention, in which they rated their achievement of recruitment skills taught in BRIEF. Research team members also completed a final study interview to provide feedback on the intervention components, training content, and use of skills in practice. RESULTS: All nine research team members completed all components of BRIEF. Survey responses showed moderate to low satisfaction with previous recruitment training before BRIEF and high satisfaction with the BRIEF training. Self-assessments showed significant increases in reported partnership with bedside nursing (p&#x2009;=&#x2009;0.02) and confirmation of family names (p&#x2009;=&#x2009;0.05) after BRIEF training. Interviews provided further evidence of overall satisfaction with the BRIEF training, its content, and the skills learned, as well as opportunities for improvement, particularly in supporting challenging conversations. CONCLUSION: This pilot study demonstrated the feasibility and acceptability of the BRIEF intervention, as well as opportunities for improvement in future training. KEY POINTS: &#xb7; It was feasible to implement the BRIEF researcher training in a single-site NICU trial.. &#xb7; BRIEF training was acceptable to research team members.. &#xb7; BRIEF training shows potential to improve relationship-based research communication..

Humans↗

Strategies for addressing bullying with the school-age child who stutters.

This article outlines basic principles for helping school-age children who stutter to deal with bullying. Bullying may affect children's school performance and feelings of self-worth and lead to depression and feelings of helplessness and loneliness. Bullying can also exacerbate stuttering behavior, increase negative emotions and negative thoughts, and reduce therapy progress. A variety of techniques can be implemented as part of the overall treatment process to help children deal with bullying. These techniques can improve children's overall success in treatment by helping them develop socially appropriate responses to bullying, reduce or prevent negative emotion that may result from stuttering, and feel more comfortable in applying speech management skills learned in treatment.

Child↗

Fostering generalization and maintenance in school settings.

One of the most challenging aspects of therapy for school-age children who stutter is generalizing the skills learned in the therapy room to other settings such as the classroom, lunchroom, playground, or home. An additional challenge is seen in maintaining gains over the long term. This article reviews common roadblocks to generalization and maintenance, including the goals of therapy, the nature of the treatment strategies that require generalization, the scheduling and implementation of generalization activities within the overall therapy process, and the child's understanding of the treatment goals. Specific strategies for overcoming these roadblocks include: desensitizing children to both stuttering and treatment strategies designed to improve fluency, using hierarchies as a way of structuring treatment and moving children toward success in their daily activities, integrating the child's real world and clinical settings, and using structured practice activities to help children solidify all of the lessons they learn in treatment. This article highlights the importance of taking a broad-based view of stuttering to help children improve their overall communication across a variety of settings and over time.

Child↗

Relevance of basic laboratory and clinical research activities as part of the vascular surgery fellowship: an assessment by program directors and postfellowship surgeons.

INTRODUCTION: Decreased federal monies for graduate medical education, increased clinical training demands, and a decreased pool of general surgery trainees applying to vascular surgery fellowships have brought into question the relevance of the fellowship research experience. This study sought to describe the recent laboratory experience of the fellows, the value of this experience to program directors (PDs) and the trainees, and what factors related to this experience contributed to the trainee entering an academic career versus a private practice career. METHODS: A survey regarding the relevance of research experience during fellowship training was mailed in 2001 to all Accreditation Council for Graduate Medical Education-approved vascular surgery fellowship PDs and vascular surgery fellows (VSFs) from 1988 to 2000 applying for the American Board of Surgery Certificate of Added Qualification in General Vascular Surgery. RESULTS: Survey responses were received from 89% of the PDs (74/83) and 69% of the VSFs (259/378). Among the PDs, 70% had completed an approved fellowship, and current bench research was performed by 46%. The PDs afforded protected research time to 69% of the VSFs (with a mean duration of 12 months). This research was in the basic science laboratory 34% of the time. Only 42% of the PDs considered basic laboratory research to be an important part of the fellowship, whereas 99% believed that clinical research was important. Among the PDs, 42% believed that more practice-oriented fellowships with no basic research were needed, whereas 35% believed that basic research should remain an integral component of the fellowship. VSF basic science productivity was significantly greater from those programs that offered protected research time as compared with those that did not (mean basic science paper published, 1.7 +/- 0.1 versus 0.3 +/- 0.6 per VSF; P <.001). At the time of the survey, 99 VSFs had entered academic careers and 136 were in private practice. Basic science research had been undertaken by 56% of the VSFs during medical school and by 53% during general surgery residency. Research during the fellowship was performed by 65% of the VSFs. This experience was considered helpful in choosing an academic or private practice career by 44% of the VSFs. A greater proportion of academic surgeons had research experience as VSFs when compared with VSFs who became private practitioners (71% versus 57%; P <.05). VSFs who entered academic careers had a more productive publication record in fellowship than did those who chose private practice (mean paper, 2.4 versus 1.5; P <.05). Overall, 78% of the VSFs believed that their research experience was maturing beyond the technical skills learned. CONCLUSION: This report provides a benchmark of the vascular surgery fellowship research experience. Most VSFs considered the research experience as it now exists to be worthwhile, and less than half of the PDs believed that it should remain as it is. Research experience in fellowship seemed more influential than that in medical school or general surgical residency in promoting an academic career.

Biomedical Research↗