Compensatory Strategy Intervention: What Older Patients Want and Why.

OBJECTIVE: Compensatory strategies can improve performance of instrumental activities of daily living in people with cognitive impairment. This study investigated patient interest in compensatory strategy interventions and preference for various intervention formats. METHODS: Semi-structured qualitative interviews with 38 older adults with cognitive impairment queried motivation to improve strategy use and interest in intervention formats/delivery methods. Two coders used thematic analysis to determine rates of interest in each intervention type and explore patient-reported barriers and facilitators to motivation and intervention models. RESULTS: Most of the samples reported motivation to enhance compensatory strategy use. Degree of motivation was driven by current experiences with strategy use, perceived benefit of potential changes, intrinsic desire to improve life and self, and current perceived need. The vast majority were interested in hour-long, multi-session, instructor-led interventions. Just over half of the sample was interested in a self-directed virtual program, and just under half was interested in involving family/friends. Facilitators and barriers to interest in intervention formats and delivery methods varied based on participants' previous experiences, preferred learning style, content, and time commitment of the intervention, and perceived current need for intervention. One-fifth of the sample expressed no interest in any intervention type, though they expressed openness to assistance in the future as needed. CONCLUSIONS: Older adults with cognitive impairment are generally motivated to enhance their compensatory strategy use. Clinicians/researchers designing compensatory strategy interventions should consider instructor-led formats, present individualized benefits of interventions, and demonstrate the benefits of both preventative and remedial intervention to optimize patient engagement.

Airway Disease in Children with Primary Ciliary Dyskinesia: Impact of Ciliary Ultrastructure Defect and Genotype.

Rationale: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance, recurrent respiratory infections, progressive airway damage, and obstructive lung disease. Although the association of ciliary ultrastructure defect/genotype with the severity of airflow obstruction has been well characterized, their association with airway abnormalities on chest computed tomography (CT) has been minimally evaluated. Objectives: We sought to delineate the association of ciliary defect class/genotype with chest CT scores in children with PCD. Methods: Cross-sectional analysis of children with PCD (N = 146) enrolled in a prospective multicenter observational study, stratified by defect type: outer dynein arm (ODA), ODA/inner dynein arm (IDA), IDA/microtubular disorganization (MTD), and normal/near normal ultrastructure with associated genotypes. CTs were scored using the MERAGMA-PCD (Melbourne-Rotterdam Annotated Grid Morphometric Analysis for PCD), evaluating airway abnormalities in a hierarchical order: atelectasis, bronchiectasis, bronchial wall thickening, and mucus plugging/tree-in-bud opacities. The volume fraction of each component was expressed as the percentage of total lung volume. The percentage of disease was computed as the sum of all components. Regression analyses were used to describe the association between clinical predictors and CT scores. Results: Acceptable chest CTs were obtained in 141 children (71 male): 57 ODA, 20 ODA/IDA, 40 IDA/MTD, and 24 normal/near normal. The mean (standard deviation) age was 8.5 (4.6) years, forced expiratory volume in 1 second (FEV1) percent predicted was 82.4 (19.5), and %Disease was 4.6 (3.5). Children with IDA/MTD defects had a higher %Disease compared with children with ODA defects (2.71% higher [95% confidence interval (CI), 1.37-4.06; P < 0.001]), driven by higher %Mucus plugging (2.35% higher [1.43-3.26; P < 0.001]). Increasing age, lower body mass index, and lower FEV1 were associated with a higher %Disease (0.23%; 95% CI, 0.11-0.35; P < 0.001 and 0.03%; 95% CI, 0.01-0.04; P = 0.008 and 0.05%; 95% CI, 0.01-0.08; P = 0.011, respectively). Conclusions: Children with IDA/MTD defects had significantly greater airway disease on CT, primarily mucus plugging, compared with children with ODA defects.

Laterality Defects in Primary Ciliary Dyskinesia: Relationship to Ultrastructural Defect or Genotype.

Rationale: The association between organ laterality abnormalities and ciliary ultrastructural defect or genotype in primary ciliary dyskinesia is poorly understood. Objectives: To determine if there is an association between presence and/or type of laterality abnormality and ciliary ultrastructural defect or genotype. Methods: Participants with primary ciliary dyskinesia in a multicenter, prospective study were grouped based on ciliary ultrastructural defect or genotype. In a retrospective analysis of these data, the association of ciliary ultrastructural defect or genotype and likelihood of a laterality abnormality was evaluated by logistic regression adjusted for presence of two loss-of-function versus one or more not-loss-of-function variants. Results: Of 559 participants, 286 (51.2%), 215 (38.5%), and 58 (10.4%) were identified as having situs solitus, situs inversustotalis, and situs ambiguus, respectively; heterotaxy, defined as situs ambiguus with complex cardiovascular defects, was present in 14 (2.5%). Compared with the group with inner dynein arm defects with microtubular disorganization, laterality defects were more likely in the outer dynein arm defects group (odds ratio [OR], 2.07; 95% confidence interval [CI], 1.21-3.54; P < 0.01) and less likely in the normal/near normal ultrastructure group (OR, 0.04; 95% CI, 0.013-0.151; P < 0.01). Heterotaxy was present in 11 of 242 (4.5%) in the outer dynein arm defects group but 0 of 96 in the inner dynein arm defects with microtubular disorganization group (P = 0.038). Conclusion: In primary ciliary dyskinesia, risk of a laterality abnormality differs by ciliary ultrastructural defect. Pathophysiologic mechanisms underlying these differences require further exploration.

Airway Inflammation in Children with Primary Ciliary Dyskinesia.

Rationale: The role of airway inflammation in disease pathogenesis in children with primary ciliary dyskinesia (PCD) is poorly understood. Objectives: We investigated relationships between sputum inflammation measurements, age, lung function, bronchiectasis, airway infection, and ultrastructural defects in children with PCD. Methods: Spontaneously expectorated sputum was collected from clinically stable children and adolescents with PCD ages 6 years and older participating in a multicenter, observational study. Sputum protease and inflammatory cytokine concentrations were correlated with age, lung function, and chest computed tomography measures of structural lung disease, whereas differences in concentrations were compared between ultrastructural defect categories and between those with and without detectable bacterial infection. Results: Sputum from 77 children with PCD (39 females [51%]; mean [standard deviation] age, 13.9 [4.9] yr; mean [standard deviation] forced expiratory volume in 1 second [FEV1]% predicted, 80.8 [20.5]) was analyzed. Sputum inflammatory marker measurements, including neutrophil elastase activity, IL-1ß (interleukin-1ß), IL-8, and TNF-α (tumor necrosis factor α) concentrations, correlated positively with age, percentage of bronchiectasis, and percentage of total structural lung disease on computed tomography, and negatively with lung function. Correlations between neutrophil elastase concentrations and FEV1% predicted and percentage of bronchiectasis were -0.32 (95% confidence interval, -0.51 to -0.10) and 0.46 (0.14 to 0.69), respectively. Sputum neutrophil elastase, IL-1ß, and TNF-α concentrations were higher in those with detectable bacterial pathogens. Participants with absent inner dynein arm and microtubular disorganization had similar inflammatory profiles compared with participants with outer dynein arm defects. Conclusions: In this multicenter pediatric PCD cohort, elevated concentrations of sputum proteases and cytokines were associated with impaired lung function and structural damage as determined by chest computed tomography, suggesting that sputum inflammatory measurements could serve as biomarkers in PCD.

A pilot study of cognition and creativity among persons with HIV disease referred for neuropsychological evaluation.

Creativity can help people to innovate, overcome obstacles, and successfully navigate challenges in daily life. Some aspects of creativity rely on the prefrontostriatal loops and executive functions, which can be compromised in persons with HIV (PWH). This pilot study examined whether neuropsychological functioning plays a role in creativity in PWH. A consecutive series of 41 PWH who were referred to an urban neuropsychology clinic in southeastern Texas were enrolled. Participants completed the Abbreviated Torrance Test for Adults (ATTA) to measure creativity, from which standardized creativity scores of fluency, originality, elaboration, and flexibility were derived. Participants also completed several measures of everyday functioning and a brief clinical neuropsychological battery measuring executive functions, motor skills, memory, and visuoconstruction. Global neuropsychological functioning showed a large, positive association with ATTA creativity performance that did not vary meaningfully by creativity domain and was independent of premorbid IQ. ATTA creativity scores were not associated with any measure of everyday functioning. Findings from this pilot study suggest that higher levels of neuropsychological functioning may support multiple dimensions of creativity in adults with HIV disease. Future studies might examine whether creativity moderates the association between HIV-associated neurocognitive impairment and various health behaviors (e.g., adherence, appointment attendance).

The Impact on Parents of Diagnosing PCD in Young Children.

Primary ciliary dyskinesia (PCD) is an incurable, rare, inherited, chronic condition. Treatment includes the regular clearing of airway mucus, aggressive treatment of infections and management of hearing loss. Caregiver burden has not been explored, hence we interviewed 18 mothers and 6 fathers of children under 6 years to understand the impact of diagnostic testing and implications of a positive diagnosis. Interviews were transcribed and thematically analysed and five key themes were identified. These included the parents' experiences following child's diagnosis, impact of child's treatment regimen on parent, impact of child's health status on parent, parent's coping strategies, and parental concerns for the future. Parents described their diagnostic journey, with the findings revealing how a lack of awareness among clinicians of the PCD symptom pattern can lead to a delayed diagnosis. Parents discussed the emotional and practical impact of a PCD diagnosis and the coping strategies employed to deal with challenges arising following a diagnosis. Parents use a variety of different lifestyle changes to accommodate their child's treatment regimen and to cope with disruptive life events such as the COVID-19 pandemic. This study provides valuable insights into parental adjustment and adaptation to a PCD diagnosis and management regimen. Going forward, this research highlights the need for integrated social care for PCD patients and their families.

Factor Structure of the Memory for Intentions Test (MIsT): A Conceptual Replication in Older Adults and People with HIV Disease.

OBJECTIVE: The Memory for Intentions Test (MIsT) is a clinical measure of prospective memory that has strong evidence for convergent, discriminative, and ecological validity. This study uses a conceptual replication design to evaluate the latent structure of the MIsT in two parallel samples who commonly experience prospective memory deficits: older adults and people living with HIV disease. PARTICIPANTS AND METHODS: Study participants included 303 people with HIV disease (ages 18-67) and 267 community-dwelling older adults (ages 50-91). Confirmatory factor analyses of the MIsT were conducted separately in each sample. We evaluated a one-factor model, as well as three two-factor models with the MIsT items loading onto each factor based on cue type, delay interval, or response modality. RESULTS: The one-factor model provided the best (and most parsimonious) fit to the data in both study samples. All two-factor models also demonstrated good fit statistics, although correlations between the two factors in each model were high and none of the two-factor models provided a significantly better fit than the one-factor model. CONCLUSIONS: Results of this conceptual replication study provide support for a robust factor structure of the MIsT across older adults and people with HIV disease. A total score for the MIsT provides the most parsimonious solution, although available evidence and theory also support the potential use of subscales (e.g., cue type). Future studies of the MIsT would be useful to determine its psychometrics in different clinical populations and across demographic factors (e.g., race/ethnicity).

Association of Neonatal Hospital Length of Stay with Lung Function in Primary Ciliary Dyskinesia.

Rationale: Primary ciliary dyskinesia (PCD), an inherited lung disease, is characterized by abnormal ciliary function leading to progressive bronchiectasis. There is wide variability in respiratory disease severity at birth and later in life. Objectives: To evaluate the association between neonatal hospital length of stay (neonatal-LOS) and supplemental oxygen duration (SuppO2) with lung function in pediatric PCD. We hypothesized that longer neonatal-LOS and SuppO2 are associated with worse lung function (i.e., forced expiratory volume in 1 second percent predicted [FEV1pp]). Methods: We performed a secondary analysis of the Genetic Disorders of Mucociliary Clearance Consortium prospective longitudinal multicenter cohort study. Participants enrolled, during 2006-2011, were <19 years old with a confirmed PCD diagnosis and followed annually for 5 years. The exposure variables were neonatal-LOS and SuppO2, counted in days since birth. The outcome, FEV1pp, was measured annually by spirometry. The associations of neonatal-LOS and SuppO2 with FEV1pp were evaluated with a linear mixed-effects model with repeated measures and random intercepts, adjusted for age and ciliary ultrastructural defects. Results: Included were 123 participants (male, 47%; mean enrollment age, 8.3 yr [range, 0 to 18 yr]) with 578 visits (median follow-up, 5 yr). The median neonatal-LOS was 9 d (range, 1 to 90 d), and median SuppO2 was 5 d (range, 0 to 180 d). Neonatal-LOS was associated with worse lung function (-0.27 FEV1pp/d [95% confidence interval, -0.53 to -0.01]; P = 0.04). SuppO2 was not associated with lung function. Conclusions: Neonatal-LOS is associated with worse lung function in pediatric PCD, independent of age and ultrastructural defects. Future research on the mechanisms of neonatal respiratory distress and its management may help us understand the variability of lung health outcomes in PCD.

The complementary utility of cognitive testing and the medication management ability assessment in older adults.

OBJECTIVE: Older adults are susceptible to cognitive declines that may limit independence. Though neuropsychologists opine about risk of functional decline, the degree to which cognitive testing and in-office simulations approximate everyday behavior is unclear. We assessed the complementary utility of cognitive testing and the face-valid Medication Management Ability Assessment (MMAA) to predict medication management among older adults. METHOD: This was a retrospective study of 234 older adults (age = 72 ± 7.7 years; 59% women) who completed the MMAA during outpatient neuropsychological evaluations. Based on comprehensive clinical assessment, most participants (n = 186) were independent in medication management, while 48 received assistance. Demographically adjusted composite scores were derived for attention/processing speed (A/PS), executive functioning (EF), visuospatial/constructional ability (VC), language, and memory domains. Univariate differences in cognition were examined across Assisted versus Independent groups. Logistic regression assessed which cognitive domains independently predicted group status. The incremental value of the MMAA was assessed, holding uniquely associated cognitive test scores constant. RESULTS: Those receiving assistance with medication management performed worse across all neurocognitive domains and the MMAA compared with independent counterparts. EF was the only unique cognitive predictor of medication management status. When modeled alone, EF and MMAA performance correctly classified 79.5% and 80.8% of cases, respectively. When modeled together, both were independently associated with medication management status and correctly classified 83.3% of cases. CONCLUSIONS: EF uniquely predicted medication management status beyond other cognitive domains. The MMAA provided complementary predictive utility. Concurrent interpretation of executive functioning and MMAA performance is advised when assessing older adults suspected of medication mismanagement. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Expression of a Truncated Form of ODAD1 Associated with an Unusually Mild Primary Ciliary Dyskinesia Phenotype.

Primary ciliary dyskinesia (PCD) is a rare lung disease caused by mutations that impair the function of motile cilia, resulting in chronic upper and lower respiratory disease, reduced fertility, and a high prevalence of situs abnormalities. The disease is genetically and phenotypically heterogeneous, with causative mutations in > 50 genes identified, and clinical phenotypes ranging from mild to severe. Absence of ODAD1 (CCDC114), a component of the outer dynein arm docking complex, results in a failure to assemble outer dynein arms (ODAs), mostly immotile cilia, and a typical PCD phenotype. We identified a female (now 34 years old) with an unusually mild clinical phenotype who has a homozygous non-canonical splice mutation (c.1502+5G>A) in ODAD1. To investigate the mechanism for the unusual phenotype, we performed molecular and functional studies of cultured nasal epithelial cells. We demonstrate that this splice mutation results in the expression of a truncated protein that is attached to the axoneme, indicating that the mutant protein retains partial function. This allows for the assembly of some ODAs and a significant level of ciliary activity that may result in the atypically mild clinical phenotype. The results also suggest that partial restoration of ciliary function by therapeutic agents could lead to significant improvement of disease symptoms.

Neurocognitive performance differences between black and white individuals with HIV disease are mediated by health literacy.

Objective:Health disparities are evident for Black Americans with HIV disease, who are disproportionally affected by the epidemic in the United States. The current study investigated whether the higher rates of neurocognitive impairment in Black Americans with HIV disease may be at least partly attributable to health literacy, which is a potentially modifiable factor. Method: Participants were 61 White and 25 Black participants (ages 27-70) with HIV disease who were enrolled in studies at an urban academic center in Southern California. Neurocognitive function was assessed by an age-adjusted global score from the Cogstate battery. Health literacy was measured by a composite score derived from the Rapid Estimate of Adult Literacy in Medicine, Newest Vital Sign, and 3-Brief. Results: Bootstrap confidence interval mediation analyses showed that health literacy was a significant mediator of the relationship between race and neurocognition; that is, there were no direct ethnoracial differences in neurocognition after accounting for health literacy. A follow-up model to confirm the directionality of this association demonstrated that neurocognition was not a significant mediator of the relationship between race and health literacy. Conclusions: Low health literacy may contribute to the higher rates of neurocognitive impairment for Black Americans with HIV disease. Future studies might examine the possible mechanism of this mediating relationship (e.g., access to health information, health behaviors, socioeconomics) and determine whether culturally tailored interventions that improve health literacy also confer broader brain health benefits for Black Americans with HIV disease.

Gender disparities in the author bylines of articles published in clinical neuropsychology journals from 1985 to 2019.

Objective: Women are becoming more prevalent in clinical neuropsychology, but gender bias and disparities persist across multiple professional domains. This study examined potential gender disparities in historical authorship trends across commonly read journals in clinical neuropsychology. Method: Analyses were conducted on 10,531 articles published in six clinical neuropsychology journals from 1985 to 2019. Each author was coded as either a man or a woman using the OpenGenderTracking Project database. Results: On average, women comprised 43.3% (±30.6) of the authors listed in clinical neuropsychology article bylines and were lead and/or corresponding author on 50.3% of these papers. Findings varied by journal, with Child Neuropsychology having the best representation of women across several study metrics. Women comprised an increasing proportion of authors over time and the gender gap in clinical neuropsychology is smaller than was recently reported for the broader field of psychology; nevertheless, the recent rates of women as authors lag behind the prevalence of women in clinical neuropsychology. Encouragingly, gender was not associated with the number of times an article was cited. Articles that included women in leadership roles had significantly more authors overall and specifically more women authors. Conclusions: Women are under-represented as authors in clinical neuropsychology journals, but they are becoming more common and their papers are cited just as frequently as men. Efforts to increase women as research mentors and sponsors may help to further close the publishing gender gap in clinical neuropsychology.

Longitudinal declines in event-based, but not time-based, prospective memory among community-dwelling older adults.

Age-related deficits in prospective memory (PM) are well established, but it is not known whether PM is stable over time among older adults. In this study, 271 community-dwelling older adults underwent abaseline neuropsychological evaluation and up to three follow-up visits, approximately 2.4 years apart. Mixed effects linear longitudinal models revealed small, but significant linear declines and between-subjects variability in event-based PM performance. There were no changes in performance on measures of time-based PM, retrospective memory, or executive functions. Changes in event-based PM were not associated with age, retrospective memory, executive functions, or everyday functioning. Among older adults, event-based PM appears to be more susceptible to linear declines than does time-based PM, which future research might examine with regard to the possible underlying cognitive mechanisms of cue encoding, monitoring, detection, and retrieval processes.

Future and past autobiographical memory in persons with HIV disease.

Objective: While HIV disease is associated with impairment in declarative memory, the ability of people with HIV (PWH) to describe past and future autobiographical events is not known. Method: Participants included 63 PWH and 28 seronegative individuals ages 50-78 who completed standardized neurocognitive and everyday functioning assessments. Participants described four events from the recent past and four imagined events in the near future, details from which were classified as internal or external to the main event. Result: PWH produced fewer autobiographical details with small-to-medium effect sizes but did not differ from seronegative participants in meta-cognitive ratings of their performance. Performance of the study groups did not vary across past or future probes or internal versus external details; however, within the entire sample, past events were described in greater detail than future events, and more external than internal details were produced. Within the PWH group, the production of fewer internal details for future events was moderately associated with poorer prospective memory, executive dysfunction, and errors on a laboratory-based task of medication management. Conclusion: Older PWH may experience difficulty generating autobiographical details from the past and simulated events in the future, which may be related to executive dyscontrol of memory processes. Future studies might examine the role of future thinking in health behaviors such as medication adherence and retention in healthcare among PWH. (PsycInfo Database Record (c) 2021 APA, all rights reserved).

How did individual differences in neurocognition and health literacy influence the initial uptake and use of health-related information about COVID-19?

Introduction: The rapid development of coronavirus disease 2019 (COVID-19) into a pandemic required people to quickly acquire, evaluate, and apply novel complex health-related information about the virus and transmission risks. This study examined the potentially unique and synergistic roles of individual differences in neurocognition and health literacy in the early uptake and use of COVID-19 public health information.Method: Data were collected between April 23 and 21 May 2020, a period during which 42 out of 50 states were under a stay-at-home order. Participants were 217 healthy adults who completed a telephone-based battery that included standard tests of neurocognition, health literacy, verbal IQ, personality, and anxiety. Participants also completed measures of COVID-19 information-seeking skills, knowledge, prevention intentions, and prevention behaviors.Results: A series of hierarchical multiple regressions with data-driven covariates showed that neurocognition (viz, episodic verbal memory and executive functions) was independently related to COVID-19 knowledge (e.g. symptoms, risks) at a medium effect size, but not to information-seeking skills, prevention intentions, or prevention behaviors. Health literacy was independently related to all measured aspects of COVID-19 health information and did not interact with neurocognition in any COVID-19 health domain.Conclusions: Individual differences in neurocognition and health literacy played independent and meaningful roles in the initial acquisition of knowledge related to COVID-19, which is a novel human health condition. Future studies might examine whether neurocognitive supports (e.g. spaced retrieval practice, elaboration) can improve COVID-19-related knowledge and health behaviors in vulnerable populations.

A Clinical Pilot Study of Spaced Retrieval Practice with a Self-Generation Booster to Improve Health-Related Memory in Persons With HIV Disease.

OBJECTIVE: Spaced retrieval practice (SRP) and self-generation are among the most replicated and effective mnemonic strategies in the cognitive psychology literature, but their benefits have not yet been realized in healthcare settings. This study used a randomized, between-subjects design to examine the hypothesis that SRP with a self-generation booster can improve memory for health-related information among clinically referred persons with HIV (PWH), who often have difficulty acquiring new health knowledge. METHOD: A consecutive series of 41 PWH referred to a county-funded urban neuropsychology clinic were enrolled. Participants were randomly assigned to learn four statements about the treatment of a mock infectious disease in either a massed study control condition (n = 20) or an SRP condition (n = 21) in which they received two distributed free recall training tests supplemented with self-generation for missed items. The primary outcome was participants' free recall of the four treatment statements after a 20-minute delay filled with nonverbal tests. RESULTS: PWH participants in the SRP condition were four times more likely than controls to recall at least one treatment statement at the 20-minute delay. SRP was not related to post-test recognition or health-related decision-making performance but was associated with moderately better self-efficacy for decision-making. CONCLUSIONS: Findings from this pilot study show the potential of SRP with a self-generation booster to improve learning and memory for health-related information among PWH in clinic.

Psychometrics and Validity of the Survey of Memory-Related Quality of Life in HIV Disease.

OBJECTIVE: Retrospective and prospective memory deficits are associated with lower quality of life (QoL); however, there are no validated measures that comprehensively and directly assess the impact of memory problems on QoL. The Survey of Memory-Related Quality of Life (SMRQoL) was developed as a 30-item questionnaire to measure memory-related QoL. METHOD: Both HIV+ (n = 195) and HIV- (n = 146) participants completed the SMRQoL, a neurocognitive research battery, and validated self-report questionnaires of memory, QoL, and mood. Participants were recruited into younger (age ≤ 40 years) and older (age ≥ 50 years) groups per the parent study design. RESULTS: The SMRQoL had a unidimensional factor structure and demonstrated measurement invariance across the HIV+ and HIV- participants. Analyses of 111 clinically stable participants (e.g., persons with no incident or remitting central nervous system disorders) who returned for a 14-month follow-up visit indicated that the SMRQoL had adequate test-retest stability. There was a significant interaction of age and HIV status on the SMRQoL, such that older HIV+ participants reported the lowest memory-related QoL. SMRQoL scores were associated with validated measures of mental and physical QoL, self-reported memory and cognitive symptoms, and performance-based memory and executive functions. CONCLUSIONS: The SMRQoL shows evidence of reliability and validity as a measure of memory-related QoL that can be used to assess the impact of memory problems on everyday life, but future work is needed to demonstrate the measure's incremental value in the context of diagnosis and treatment.

Sinus Development and Pneumatization in a Primary Ciliary Dyskinesia Cohort.

BACKGROUND: Primary ciliary dyskinesia (PCD) is a genetically diverse disease which causes impaired mucociliary clearance, and results in pulmonary, otologic, and rhinologic disease in affected patients. Genetic mutations in multiple genes impair the ability of patients to clear mucous from the lungs, middle ear, and sinonasal cavity and lead to chronic pulmonary and sinonasal symptoms. METHODS: We identified 17 PCD patients who had available CT scans. Volumes for bilateral maxillary, sphenoid, and frontal sinuses were calculated. A control population of patients who had preoperative CT scans for endoscopic endonasal resection of skull base pathology without sinonasal cavity involvement was also identified. RESULTS: The mean age of PCD was 33 and ranged from 13 to 54 years. Patients were age- and gender-matched to a control group that underwent resection of anterior skull-base tumors and had a mean age of 35 that ranged between 17-53 years old. The volumes for all thee sinus cavities were significantly smaller (p < 0.007) compared to the control population. The average Lund-Mackay score was 10.6 in the PCD cohort (range 6-16) in comparison to an average of 0.7 in the control cohort (range 0-2). CONCLUSIONS: Overall sinus volumes were smaller in patients with PCD compared to our control population. Future studies will be aimed at understanding defects in sinus development as a function of specific genetic mutations in PCD patients. Ultimately, a better understanding of the underlying pathophysiology of PCD will allow us to identify the optimal treatment practices for this unique patient group.

Verbal Learning Mediates the Relationship Between Executive Functions and a Laboratory Task of Medication Management in HIV Disease.

OBJECTIVE: Impairments in executive functions and learning are common in HIV disease and increase the risk of nonadherence to antiretroviral therapy. The mixed encoding/retrieval profile of HIV-associated deficits in learning and memory is largely driven by dysregulation of prefrontal systems and related executive dysfunction. This study tested the hypothesis that learning may be one pathway by which executive dysfunction disrupts medication management in people living with HIV (PLWH). METHOD: A total of 195 PLWH completed a performance-based laboratory task of medication management capacity and clinical measures of executive functions, verbal learning and memory, and motor skills. RESULTS: Executive functions were significantly associated with verbal learning and medication management performance. In a model controlling for education, learning significantly mediated the relationship between executive functions and medication management, and this mediation was associated with a small effect size. In particular, executive dysfunction was associated with diminished use of higher-order learning strategies. Alternate models showed that executive functions did not mediate the relationship between learning and medication management nor did motor skills mediate the relationship between executive functions and medication management. CONCLUSIONS: PLWH with executive dysfunction may demonstrate difficulty in learning new information, potentially due to ineffective strategy use, which may in turn put them at a higher risk for problems managing their medications in the laboratory. Future studies may wish to investigate whether compensatory neurocognitive training (e.g., using more effective learning strategies) may improve medication management among PLWH.

Clinical identification of psychogenic nonepileptic events using combinations of psychological tests in a veteran sample.

OBJECTIVE: Patients with psychogenic nonepileptic events (PNEE) exhibit heterogenous symptoms and are best diagnosed with long-term video-electroencephalogram (vEEG) data. While extensive univariate data suggest psychological tests may confirm the etiology of PNEE, the multivariate discriminant utility of psychological tests is less clear. The current study aggregated likelihood ratios of multiple psychological tests to evaluate incremental and discriminant utility for PNEE. METHODS: Veterans with vEEG-diagnosed PNEE (n = 166) or epileptic seizures (n = 92) completed self-report measures and brief neuropsychological evaluations during the 4-day vEEG hospitalization. Receiver operating characteristic (ROC) curves identified discriminating psychological tests and corresponding cut-scores (0.85 minimum specificity). Likelihood ratios from the remaining cut-scores were sequentially linked using the sample base rate of PNEE (64%) and alternative base rates (10%, 20%, 30%, 40%) to estimate posttest probabilities (PTP) of test combinations. RESULTS: The Health Attitudes Survey, Health History Checklist, and Minnesota Multiphasic Personality Inventory-2-Restructured Form scales FBS-r, RC1, MLS, and NUC were identified as discriminating indicators of PNEE. Average PTPs were ≥90% when three or more indicators out of six administered were present at the sample base rate. Regardless of PNEE base rate, PTP for PNEE was ≥98% when all discriminating indicators were present and 92-99% when five of six indicators administered were present. PTPs were largely consistent with observed positive predictive values, particularly as indicators present increased. SIGNIFICANCE: Aggregating psychological tests identified PNEE with a high degree of accuracy, regardless of PNEE base rate. Combining psychological tests may be useful for confirming the etiology of PNEE.