The relationship between working memory and anxiety in individuals with early treated phenylketonuria (PKU).

OBJECTIVE: Although early diagnosis and treatment prevent the severe impairments associated with untreated phenylketonuria (PKU), individuals with early treated PKU (ETPKU) nonetheless experience significant neurocognitive and psychological sequelae, including difficulties in working memory (WM) and increased risk of anxiety. The primary objective of the present study was to examine the extent to which anxiety may moderate the relationship between ETPKU and WM performance. METHOD: A sample of 40 adults with ETPKU and a demographically comparable sample of 40 healthy adults without PKU completed a comprehensive assessment of WM performance and anxiety symptomatology. Data were collected using a variety of remote assessment methods (e.g., web-based neurocognitive tests, semistructured interview, report-based measures). RESULTS: The ETPKU group demonstrated significantly poorer WM performance as compared to the non-PKU group. The groups did not differ significantly in anxiety; however, high anxiety was more common in the ETPKU group (53% of sample) than the non-PKU group (33%). A significant interaction between anxiety, metabolic control (as reflected by Phe levels), and WM performance was observed for the ETPKU group. Individuals with high anxiety and/or high Phe levels (> 360 µmol/L) performed poorer than the non-PKU group. Individuals with low anxiety and relatively low Phe levels (< 360 µmol/L) performed comparably to the non-PKU group. CONCLUSIONS: Anxiety was found to moderate the relationship between Phe levels and WM performance in individuals with ETPKU. This finding underscores the importance of accounting for anxiety when evaluating neurocognitive performance in individuals with ETPKU whether for research or clinical purposes. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Total choline intake and working memory performance in adults with phenylketonuria.

BACKGROUND: Despite early diagnosis and compliance with phenylalanine (Phe)-restricted diets, many individuals with phenylketonuria (PKU) still exhibit neurological changes and experience deficits in working memory and other executive functions. Suboptimal choline intake may contribute to these impairments, but this relationship has not been previously investigated in PKU. The objective of this study was to determine if choline intake is correlated with working memory performance, and if this relationship is modified by diagnosis and metabolic control. METHODS: This was a cross-sectional study that included 40 adults with PKU and 40 demographically matched healthy adults. Web-based neurocognitive tests were used to assess working memory performance and 3-day dietary records were collected to evaluate nutrient intake. Recent and historical blood Phe concentrations were collected as measures of metabolic control. RESULTS: Working memory performance was 0.32 z-scores (95% CI 0.06, 0.58) lower, on average, in participants with PKU compared to participants without PKU, and this difference was not modified by total choline intake (F[1,75] = 0.85, p = 0.36). However, in a subgroup with complete historical blood Phe data, increased total choline intake was related to improved working memory outcomes among participants with well controlled PKU (Phe = 360 µmol/L) after adjusting for intellectual ability and mid-childhood Phe concentrations (average change in working memory per 100 mg change in choline = 0.11; 95% CI 0.02, 0.20; p = 0.02). There also was a trend, albeit nonsignificant (p = 0.10), for this association to be attenuated with increased Phe concentrations. CONCLUSIONS: Clinical monitoring of choline intake is essential for all individuals with PKU but may have important implications for working memory functioning among patients with good metabolic control. Results from this study should be confirmed in a larger controlled trial in people living with PKU.

Brief Report: Flanker Visual Filtering Ability in Older Adolescents with Autism Spectrum Disorder.

Recent research has documented impaired ability to resist interference from visual distractors in individuals with autism spectrum disorder (ASD) and suggests that this phenomenon may be more pronounced in young versus older children (Christ et al., Neuropsychology 25(6):690-701, 2011). The present study extends previous findings by examining visual filtering inhibitory ability within an older adolescent population. A flanker visual filtering task was administered to 36 adolescents with ASD and 44 adolescents without ASD (age: 11-20 years). Analysis revealed no evidence of group differences in visual filtering performance. Taken together with previous research, these results suggest that during early adolescence the previously observed impairment may resolve or compensatory strategies develop, allowing individuals with ASD to perform as well as their neurotypical peers.

Statistical Learning and Spelling: Older Prephonological Spellers Produce More Wordlike Spellings Than Younger Prephonological Spellers.

The authors analyzed the spellings of 179 U.S. children (age = 3 years, 2 months-5 years, 6 months) who were prephonological spellers, in that they wrote using letters that did not reflect the phonemes in the target items. Supporting the idea that children use their statistical learning skills to learn about the outer form of writing before they begin to spell phonologically, older prephonological spellers showed more knowledge about English letter patterns than did younger prephonological spellers. The written productions of older prephonological spellers were rated by adults as more similar to English words than were the productions of younger prephonological spellers. The older children s spellings were also more wordlike on several objective measures, including length, variability of letters within words, and digram frequency.

A multiscale analysis of gene flow for the New England cottontail, an imperiled habitat specialist in a fragmented landscape.

Landscape features of anthropogenic or natural origin can influence organisms' dispersal patterns and the connectivity of populations. Understanding these relationships is of broad interest in ecology and evolutionary biology and provides key insights for habitat conservation planning at the landscape scale. This knowledge is germane to restoration efforts for the New England cottontail (Sylvilagus transitionalis), an early successional habitat specialist of conservation concern. We evaluated local population structure and measures of genetic diversity of a geographically isolated population of cottontails in the northeastern United States. We also conducted a multiscale landscape genetic analysis, in which we assessed genetic discontinuities relative to the landscape and developed several resistance models to test hypotheses about landscape features that promote or inhibit cottontail dispersal within and across the local populations. Bayesian clustering identified four genetically distinct populations, with very little migration among them, and additional substructure within one of those populations. These populations had private alleles, low genetic diversity, critically low effective population sizes (3.2-36.7), and evidence of recent genetic bottlenecks. Major highways and a river were found to limit cottontail dispersal and to separate populations. The habitat along roadsides, railroad beds, and utility corridors, on the other hand, was found to facilitate cottontail movement among patches. The relative importance of dispersal barriers and facilitators on gene flow varied among populations in relation to landscape composition, demonstrating the complexity and context dependency of factors influencing gene flow and highlighting the importance of replication and scale in landscape genetic studies. Our findings provide information for the design of restoration landscapes for the New England cottontail and also highlight the dual influence of roads, as both barriers and facilitators of dispersal for an early successional habitat specialist in a fragmented landscape.

Toward consensus-based actions that balance invasive plant management and conservation of at-risk fauna.

Limiting the spread of invasive plants has become a high priority among natural resource managers. Yet in some regions, invasive plants are providing important habitat components to native animals that are at risk of local or regional extirpation. In these situations, removing invasive plants may decrease short-term survival of the at-risk taxa. At the same time, there may be a reluctance to expand invaded habitats to benefit at-risk species because such actions may increase the distribution of invasive plants. Such a dilemma can result in "management paralysis," where no action is taken either to reduce invasive plants or to expand habitats for at-risk species. A pragmatic solution to this dilemma may be to develop an approach that considers site-specific circumstances. We constructed a "discussion tree" as a means of initiating conversations among various stakeholders involved with managing habitats in the northeastern USA to benefit several at-risk taxa, including New England cottontails (Sylvilagus transitionalis). Major components of this approach include recognition that expanding some invaded habitats may be essential to prevent extirpation of at-risk species, and the effective control of invasive plants is dependent on knowledge of the status of invasives on managed lands and within the surrounding landscape. By acknowledging that management of invasive plants is a complex issue without a single solution, we may be successful in limiting their spread while still addressing critical habitat needs.