Search Results (320)

Amyotrophic Lateral Sclerosis (ALS) is increasingly recognized as a multisystem disorder. However, SOD1-associated ALS has traditionally been regarded as an exclusively motor phenotype. This systematic review challenges that paradigm by characterizing the cognitive and behavioural profiles of SOD1 mutation-related ALS. Following PRISMA guidelines, a systematic PubMed search identified 17 eligible studies. Across the included studies, encompassing 222 patients with SOD1 variants, three of the analyzed subjects fulfilled the diagnostic criteria for frontotemporal dementia (FTD), and a larger proportion exhibited multidomain cognitive deficits, including executive dysfunction, language impairment, and working memory decline. Behavioural disturbances, such as apathy, emotional lability, and mental rigidity, were also prominent. The historical assumption that SOD1 mutations completely spare cognitive networks likely reflects an under-recognition of subtle manifestations, compounded by the limited sensitivity of earlier screening tools. These findings indicate that cognitive and behavioural involvement can occur in SOD1-ALS, highlighting a highly heterogeneous phenotype. Encompassing varying degrees of severity—from subtle differences in neuropsychological test scores to formal FTD diagnoses—the current evidence points towards possible variant-specific phenotypes rather than a uniform cognitive syndrome. Routine multidomain cognitive screening is crucial in this population. Properly characterizing these non-motor features allows for more accurate disease staging, better monitoring of clinical progression, and the implementation of truly personalized care strategies. Full article

Climate change strongly impacts the physiological processes in the grapevine. Two Romanian autochthonous cultivars, Șarba and Fetească neagră (Vitis vinifera L.), grown under semi-arid conditions in 2024 (De Martonne Index: 23.6; Hydrothermal Coefficient: 0.75), showed different physiological behaviours. Leaf gas exchange parameters (A, g_s, E, C_i) and water use efficiency (WUE) were assessed at three phenophases (flowering, berry growth, and véraison), while photosynthetic pigment indices (Chl a/b, Chl/C+X) and leaf dry matter content (dm) were additionally determined at harvest maturity. Multivariate analysis consistently separated the two cultivars into distinct physiological groups: responses consistent with near-isohydric behaviour in Șarba and anisohydric behaviour in Fetească neagră, based on gas-exchange parameters interpreted within the established isohydric/anisohydric framework, as direct water potential measurements were not performed. Șarba exhibited a water-conserving strategy at véraison—characterised by early stomatal closure, high WUE, and maintained chlorophyll—protecting vine water status at the cost of reduced leaf carbon assimilation. Fetească neagră, by contrast, kept its stomata progressively open, sustaining high gas exchange rates in a pattern consistent with progressively declining shoot water potential. Berry sugars are expected to concentrate passively late in ripening, while severe deficit risks berry shrivelling and, under prolonged drought, premature senescence of basal leaves, to our knowledge, a phenomenon not previously reported for this cultivar. These findings support cultivar-specific management, with implications for irrigation scheduling and varietal selection under climate change. Full article

This systematic review synthesises computational models of language deficits in bilingual aphasia, focusing on code-switching and translation. Per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, this study identified 42 publications (2010–2025). Of the three deficit types, lexical retrieval and naming deficit simulations are most mature, replicating naming errors and predicting cross-language generalisation. Code-switching and translation deficit modelling is extremely limited: only a single computational lesioning study has addressed both through mechanistic simulation. Machine learning methods show preliminary promise for predicting treatment outcomes but remain largely data-driven. Three key challenges emerge: (1) methodological fragmentation—heterogeneous model types, lesion implementations, and evaluation metrics, with no standardised quantitative validation; (2) an underdeveloped theoretical foundation, as most studies are confined to behavioural fitting rather than testing hypotheses on impaired language control via computational lesions; (3) limited clinical translation, with few predictive studies integrating longitudinal patient data, with approximately 70% of studies concentrated in North America/Western Europe and biased toward English–Spanish bilinguals, limiting theoretical generalisability. Future work should shift from behavioural fitting to explainable mechanistic simulation, develop predictive frameworks for personalised rehabilitation, and establish standardised protocols bridging computational modelling with clinical application. Full article

Background/Objectives: Dental caries remains one of the most prevalent and preventable childhood diseases worldwide. Despite universal access to free dental care in Saudi Arabia, preventable oral diseases remain common, highlighting persistent gaps in oral health literacy and preventive behaviours among children. Male students consistently demonstrate poorer oral health knowledge and practices than their female counterparts, yet they remain understudied. This study aimed to assess oral health knowledge, attitudes, and practices (KAP) among male primary school students in Al Lith city, Saudi Arabia, and to identify their associated sociodemographic determinants. Methods: A school-based analytical cross-sectional study was conducted during the 2023–2024 academic year in Al Lith city, Makkah Province, Saudi Arabia. A total of 430 male students aged 10–13 years from grades five and six were recruited via multistage cluster sampling from 8 randomly selected schools. A validated, interviewer-administered, Arabic-language questionnaire assessed knowledge (18 items), attitudes (12 Likert-scale items), and practices (12 items) related to oral health, alongside sociodemographic data. The study was designed and reported in accordance with the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement. Data were analysed using IBM SPSS Statistics version 27. Descriptive statistics and multivariable logistic regression were performed. Results: Of the 430 participants, 83.3% were aged 11–12 years, 89.8% were Saudi nationals, and 89.1% resided in intact, two-parent households. Knowledge deficits were widespread: only 8.4% correctly identified the recommended timing of sweet consumption, 20.2% understood dental plaque, and 28.6% recognised the protective role of fluoride. Attitudes were generally positive, with 80.1% agreeing that teeth should be brushed twice daily and 71.6% endorsing regular dental visits; however, 60.3% held that dental attendance was necessary only when pain was present. Substantial practice gaps were also evident: only 33.3% brushed for the recommended two minutes, and 12.6% had never visited a dentist. In multivariable analysis, low household income was independently associated with better oral health knowledge (adjusted odds ratio [AOR] = 2.22; 95% confidence interval [CI]: 1.04–4.74; p = 0.039), divorced parental status was independently associated with positive oral health attitudes (AOR = 5.40; 95% CI: 1.28–22.79; p = 0.022), and older age was independently associated with poorer oral health practices (AOR = 0.72; 95% CI: 0.55–0.93; p = 0.014). Conclusions: Male primary school students in Al Lith demonstrated generally positive oral health attitudes but significant deficits in knowledge and preventive practices. Comprehensive, school-based oral health promotion programmes that address key misconceptions, actively engage parents, and integrate culturally accepted practices—such as miswak use—with evidence-based preventive guidance are needed. Full article

Introduction: Chronic inflammation in Crohn’s disease (CD) is associated with systemic alterations, including the loss of skeletal muscle mass. While nutritional deficits are known to impact clinical trajectories, the specific prognostic value of muscle wasting remains underutilised. The primary objective of this study is to construct a composite score integrating sarcopenia with paraclinical parameters to predict an unfavourable disease outcome (UO) more accurately. Materials and Methods: We conducted a retrospective study of patients with CD who were hospitalized at our institution between January 2020 and June 2024 and were treated with biologics or small molecules. Using previous CT scans, we measured skeletal muscle area (SMA) and skeletal muscle index (SMI) to assess their association with disease outcomes. Univariate and multivariate analyses were performed to identify significant predictors of UO, and Area Under the Curve (AUC) values were calculated to assess the predictive performance and to construct a composite prognostic score. Results: Multivariate analysis in the study group (52 patients) identified sarcopenia (low SMI), faecal calprotectin levels >129 μg/g, and complicated disease behaviour (Montreal B2/B3) as independent predictors of UO. Integrating these parameters into a composite risk score resulted in a promising predictive model with an Area Under the Curve (AUROC) of 0.864, significantly outperforming individual biomarkers. Conclusions: We performed a hypothesis-generating study suggesting that nutritional assessment in CD could provide an important benefit in stratifying patients for whom additional nutritional interventions may enhance the long-term outcomes. Full article

Background: Falls among older adults are linked to central nervous system deterioration and executive function deficits. Objectives: This study evaluates the feasibility of a gamified EEG-based neurofeedback intervention combined with adaptive working-memory training, targeting neural processes associated with attentional inhibition and working memory in older adults, and characterises, as exploratory secondary outcomes, the oscillatory and functional measures that change over the training period. Methods: Twenty healthy older adults ($65.0\pm 3.3$ years) completed a 24-session longitudinal training programme. The intervention combined real-time alpha-band neurofeedback (NF), targeting left-frontal alpha activity associated with inhibitory control, with an adaptive N-back task engaging theta-band working memory processes. Behavioural outcomes included the Colour Trail Making Test A and B (CTMT-A/B), the Berg Balance Scale short form (BBS-3P), and the four-item Dynamic Gait Index (DGI-4). EEG was recorded at the Early, Middle, and Later stages to characterise training-related neural and behavioural changes. Results: The programme proved highly deliverable: adherence was 100% across all 24 sessions, no session was terminated early, and no severe adverse events occurred. Participants acquired control of the trained signal, with left-frontal alpha power rising across training at the neurofeedback target site, and frontal theta increased bilaterally under adaptive working memory load, consistent with the reduced hemispheric asymmetry characteristic of this age group. Scores changed in the direction of improvement on all four behavioural measures; however, functional changes were small, and cognitive changes cannot be separated from repeated-testing effects. Of sixteen candidate EEG–behaviour associations, four showed moderate-to-large participant-level coefficients and were retained as candidate associations: right-frontal alpha with balance ($r=-0.64$) and with gait adaptability ($r=-0.48$), and bilateral frontal theta with processing speed ($r=-0.53$ and $-0.51$). Several associations that appeared strong when observations were pooled across stages did not survive participant-level modelling, indicating that repeated-measures methods are needed in this literature. All associations are exploratory, uncorrected for multiplicity, and reported with effect sizes and confidence intervals. Conclusions: The gamified neurofeedback and working-memory training programme was feasible, well tolerated, and fully adhered to in a supervised experimental setting by community-dwelling older adults and was accompanied by measurable modulation of the targeted frontal rhythms. This study delivers a shortlist of candidate EEG features, with the effect-size estimates needed to power a confirmatory trial. Because the design is single-arm, both functional scales approached ceiling, and the same test forms were repeated at each stage, practice effects cannot be separated from intervention effects, and these associations are hypothesis-generating; a sham-controlled trial in a fall-prone population is the appropriate next step. Full article

A control that cannot be audited is a control that does not yet exist operationally. Commercial off-the-shelf (COTS) Internet of Things (IoT) devices in Ambient Assisted Living (AAL) environments expose this problem sharply: they export raw behavioural telemetry rather than the security-auditable event records required by the logging and monitoring controls of ISO/IEC 27001:2022. This study formalises that deficit as the Admissibility Gap, a weighted, field-level measure of the mismatch between native device output and the evidentiary requirements of Annex A. An audit of four publicly available AAL datasets (CASAS, SPHERE, UCI HAR, OPPORTUNITY) confirms that identity attribution, integrity, firmware version, and privacy-minimisation governance fields are universally absent, establishing that the gap is systemic. To close it, this study proposes the Compliance Transformation Layer, an edge middleware applying three rules: LDAP-based identity attribution, keyed HMAC-SHA256 integrity sealing with firmware baseline injection, and privacy-preserving GPS truncation. An experimental campaign on 10,000 synthetic records reduced the weighted Admissibility Gap deficit from 57.0% to 4.7% (an illustrative figure under the authors’ weight vector; because the transformation rules apply deterministically, this is a demonstration of sufficiency rather than an independent validation, and its direction is robust to the weighting), with outputs mapped to the Microsoft Sentinel Common Event Format schema and the BSI IT-Grundschutz OPS.1.1.5 logging requirements. Benchmarking on Raspberry Pi 4 hardware yielded a mean per-record latency of 1.574 ms at idle, demonstrating that audit-ready logging is achievable from the edge gateway inward on commodity hardware without hardware or firmware modification. The integrity and identity guarantees are enforced from the point of gateway ingestion; the device-to-gateway segment is treated as a declared trust boundary. Full article

Background: Post-Traumatic Stress Disorder (PTSD) is associated with well-documented alterations in reactive brain processing of stimuli or events, whereas little is known about proactive/anticipatory neural dynamics preceding and preparing for task execution. This study aimed to investigate both proactive and reactive brain control of PTSD patients during a cognitive task, using the event-related potential (ERP) method. Methods: Twenty-one PTSD patients were matched to thirty healthy controls. Proactive ERP components, such as the Bereitschaftspotential (BP), prefrontal Negativity (pN), and visual Negativity (vN), were analysed to assess motor, cognitive, and sensory preparation, respectively. Reactive components such as the P1, N1, and P3 were also analysed to evaluate perceptual, attentional, and decisional processes, respectively. Results: Results revealed in PTSD patients a marked reduction in the amplitudes of all anticipatory components, suggesting a generalized impairment of top-down control in task preparation. Analysis of post-stimulus reactive processing revealed no group differences in early components associated with perceptual and attentional processing (P1, N1), but a reduction of the P3 in the PTSD group, indicating impaired higher-order cognitive processing related to decision-making and task closure. Behaviourally, patients exhibited longer and more variable response times, although accuracy remained unaffected, consistent with a speed-accuracy trade-off. Conclusions: To our knowledge, this is the first ERP study to examine anticipatory brain activity in PTSD through the BP, pN, and vN components. These results provide evidence of widespread proactive processing deficits in PTSD, consistent with previous reports of altered functioning within prefrontal, cingulate, and visual association networks. Moreover, anticipatory ERP components may represent promising neurophysiological markers for the assessment of cognitive dysfunction in PTSD. Full article

Conventional photovoltaic performance indicators provide useful benchmarking but do not determine whether production deficits arise from expected weather and module effects or from unresolved plant-side behaviour. This study develops a residual performance diagnostic framework for data-limited grid-connected photovoltaic plants and applies it to two adjacent fixed-tilt crystalline-silicon systems, each rated at 99.36 kW_p, in South Corfu, Greece, over 2016–2023. An expected-energy baseline was constructed using hourly irradiance and meteorological data from the Photovoltaic Geographical Information System after explicit correction for incidence-angle effects, irradiance-level response, module temperature, and first-order air-mass and spectral effects. Measured alternating-current energy delivered to the grid, obtained from the Hellenic Electricity Distribution Network Operator, was then compared with the corrected expected energy through a weather-adjusted system-efficiency indicator and its complementary residual-loss coefficient. The explicit module and weather loss envelope remained close to 9.01%, with temperature as the largest modelled component. Residual losses increased from 10.76% to 14.53% in Plant 1 and from 10.51% to 16.26% in Plant 2. The fitted apparent annual declines were 0.453 percentage points per year for Plant 1 and 0.609 percentage points per year for Plant 2. A generation-hour uncertainty analysis based on independent observations from the Hellenic National Meteorological Service gave expanded uncertainties of ±5.80% for expected energy and ±5.89% for the system-efficiency indicator. The framework is therefore presented as an uncertainty-bounded screening method for prioritizing inspection and maintenance, not as a root-cause diagnostic or a formal performance-loss-rate assessment. Full article

Background/Objectives: Adverse neurological events caused by imipenem are usually more frequent in patients with epilepsy. This study investigates the effects of imipenem on hippocampal neurogenesis, astroglial activation, and behaviour in animals with pro-epileptogenic alterations. Methods: Five-month-old Wistar rats were divided into four groups: control (CT), pentylenetetrazol (PTZ), imipenem-treated group (IM), and imipenem after PTZ-kindling (IM-PTZ). Epileptogenesis was induced by PTZ-kindling for 28 days, followed by 10 days of imipenem (40 mg/kg) or saline injections. Rats underwent memory and anxiety tests followed by immunohistochemical analyses of doublecortin (DCX) and glial fibrillary acidic protein (GFAP) in the hippocampus. Results: Both kindled groups displayed comparable impairments in spatial learning and working memory, while imipenem alone did not alter behaviour. The IM-PTZ group failed to reach control-level performance only in the final Morris Water Maze acquisition trials, suggesting a deficit in memory consolidation rather than a total loss of retention, as the probe trial remained comparable across all groups. Although PTZ increased anxiety-like behaviour, no locomotor differences were observed in open-field test, confirming cognitive impairments were not due to motor deficits. These behavioural changes mirrored distinct cellular alterations: while PTZ alone triggered a compensatory increase in DCX neuronal density and astrocytic branching, imipenem co-administration suppressed this neurogenic response, bringing DCX to baseline. Furthermore, combined treatment induced an uncoupling of astrocytic response, marked by increased astrocyte density and reduced process complexity in dentate hilus and Cornus Ammonis 3 (CA3). Conclusions: These results suggest that imipenem modulates the cellular substrate of the pre-sensitized brain, interfering with hippocampal plasticity and glial compensatory mechanisms, rather than directly driving further behavioural deterioration. Full article

Background: Attention-deficit/hyperactivity disorder (ADHD) has traditionally been understood through executive and frontostriatal models, but increasing evidence suggests that metabolic, inflammatory, circadian, and neuroendocrine mechanisms may also contribute to clinical heterogeneity. The hypothalamus is a central regulatory hub for appetite, sleep–wake organization, stress responsivity, autonomic function, and endocrine signalling. Objective: This review evaluated evidence linking ADHD with hypothalamic inflammation and related metabolic, inflammatory, circadian, and neuroendocrine dysregulation, with particular emphasis on appetite regulation, sleep and circadian function, stress reactivity, and pharmacological treatment response and tolerability. Methods: A systematic literature search was conducted in PubMed/MEDLINE, Embase, PsycINFO, Scopus, and Web of Science Core Collection from database inception to 31 May 2026. Human observational and intervention studies formed the primary evidence base. Evidence was synthesized narratively. Where available, quantitative findings from previously published meta-analyses were summarized to provide an overview of the strength and consistency of the evidence. Results: The reviewed evidence indicates that ADHD is associated with increased obesity risk, altered appetite-related hormones, immune-inflammatory changes, delayed circadian timing, atypical cortisol responsivity, and treatment-related effects on appetite, sleep, cardiovascular physiology, and tolerability. The strongest quantitative evidence concerned obesity, inflammatory markers, appetite hormones, and type 2 diabetes risk. Direct evidence for hypothalamic inflammation in ADHD remains limited, but converging indirect findings support hypothalamic and neuroendocrine mechanisms as biologically plausible contributors in a subgroup of individuals with ADHD. Conclusions: ADHD appears to be associated with broader metabolic, inflammatory, circadian, and neuroendocrine vulnerabilities beyond its core attentional and behavioural symptoms. Hypothalamic pathways should be interpreted as a mechanistic hypothesis rather than an established causal mechanism. Future longitudinal and multimodal studies are needed to clarify causality and identify biologically defined ADHD subgroups. Full article

Assessing projected hydrological scenarios from a risk perspective facilitates water governance and management. This study, framed in an informed and contextualized potential impact assessment process, evaluates the physical sensitivity to climate change on low flows and droughts, in a water supply sector, for illustrative purposes. More specifically, we evaluated 28 neutral scenarios using the WaSiM-ETH model on six catchments in Quebec, Canada. Guided by CORDEX—RCP 8.5 projections, the 28 scenarios are constructed around seven annual temperature changes ranging from 0 °C to 6 °C and four non-uniform monthly changes in precipitation ranging from −10% to +20%. The results show, for example, that for a scenario involving a 10% increase in precipitation and a 3 °C rise in temperature, the low flows reductions (7Q2) would range from 15% to more than 30%, the deficit in water volume during the droughts would increase by 2- to 3-fold, and the duration would vary little. No clear correlation was found between low flows and drought behaviour, with each catchment responding individually to the same climatic changes. This study thus advocates a tailored and context-specific methodology to operationalize risk assessment and make water resource management more efficient. Full article

Background/Objectives: To assess the nutritional knowledge and dietary intake of Gaelic team sport athletes and investigate any association between nutrition knowledge and dietary intake. Methods: Male/female athletes competing in Gaelic football, hurling and camogie, aged 18–40 years and training/competing at least twice weekly, were recruited. Nutrition knowledge was assessed using the validated Nutrition for Sport Knowledge Questionnaire (NSKQ). Dietary intake was assessed using a 4-day semi-quantitative food diary. Results: In total, 354 players (male n = 215, female n = 139; elite n = 133, sub-elite n = 221) completed the NSKQ; 53.1% had “poor” knowledge (score ≤49%). Dietary intake was reported by 139 players (39.3%), whose mean knowledge was also “poor” (49.9 ± 13.1%), with scoring patterns identical to non-reporters. Participants scored “average” in weight management, macronutrient, and alcohol subsections, and “poor” in micronutrient, sports nutrition, and supplement subsections. Dietary intake was sub-optimal; 84.9% under-consumed energy (median deficit 755 kcal/d [IQR −1085, −115]), primarily due to insufficient carbohydrate intake (median 3.2 g/kg/d [IQR 2.6, 3.8]; only 20.9% met the 4–8 g/kg/d recommendation). Most participants met protein and fat recommendations, although few met vitamin D or fibre recommendations, and no female participant met the iron recommendation. Those with average or above knowledge were more likely to meet energy and fibre recommendations; no further significant associations were observed. Conclusions: These findings suggest that Gaelic team sport athletes lack nutrition knowledge, and their dietary intake appears sub-optimal in several respects. In unadjusted analyses, higher knowledge was modestly associated with meeting energy and fibre recommendations. These findings highlight the need for nutrition interventions, combining education with behaviour change strategies. Full article

Background: Neurocutaneous syndromes (NCS), including neurofibromatosis type 1 (NF1), tuberous sclerosis complex (TSC), and Sturge–Weber syndrome (SWS), are rare neurodevelopmental disorders frequently associated with epilepsy, cognitive impairment, and behavioural difficulties. Although caused by different genetic alterations, these disorders share biological mechanisms that influence brain development, neuronal connectivity, and network excitability. Beyond being a common neurological manifestation, epilepsy is increasingly recognized as an important factor influencing cognitive and behavioural outcomes in neurocutaneous syndromes. Methods: This narrative review summarizes current evidence on the relationship between epilepsy, cognitive dysfunction, and behavioural manifestations in major neurocutaneous syndromes. Attention is given to epileptogenic mechanisms, shared molecular pathways, and factors influencing long-term neurodevelopmental outcomes. Results: Epilepsy is consistently associated with cognitive and behavioural outcomes in neurocutaneous syndromes, particularly in disorders characterized by early-onset and treatment-resistant seizures. Early seizure onset, poor seizure control, and persistent network dysfunction have been associated with intellectual disability, executive dysfunction, attention deficits, autism spectrum features, and impaired adaptive functioning. In TSC and SWS, epilepsy burden is strongly associated with cognitive outcome, particularly in interaction with underlying structural, vascular, and molecular abnormalities. In neurofibromatosis type 1, cognitive and behavioural difficulties are more often related to altered neuronal connectivity and dysregulated signalling pathways, although epilepsy may further contribute to neurodevelopmental impairment in a subset of patients. Despite their distinct genetic origins, these disorders converge on dysregulated RAS/MAPK, PI3K/AKT/mTOR, and Gαq-mediated signalling pathways that influence both epileptogenesis and brain development. Conclusions: Despite their distinct genetic origins, major neurocutaneous syndromes converge on common pathways linking epilepsy, network dysfunction, and neurodevelopmental impairment. Understanding how these processes interact may facilitate earlier intervention and more accurate prognostic assessment, ultimately improving long-term outcomes for affected children. Full article

Background. Developmental dyslexia (DD) is a neurodevelopmental disorder characterized by persistent difficulties acquiring fluent reading. A core feature is impaired phonological processing, though its etiology remains debated. Two competing accounts attribute phonological deficits either to reduced acoustic sensitivity to lexical stress cues or to insufficient cognitive support during phoneme processing. Methods. To test these accounts, 57 Italian children (28 with DD, 29 typically developing) completed a phoneme monitoring task in which targets appeared in strong or weak syllables, varying in acoustic salience. A composite acoustic salience factor was derived from target cue properties, and an individual cognitive factor was computed from IQ, working memory, and shifting attention. Pupillometry was used to assess auditory sensitivity to acoustic salience and cognitive effort in real time. Results. The results showed that, behaviourally, children with DD showed significantly lower target identification accuracy and d’-sensitivity. However, pupil dilation during target processing did not differ between the two groups, while children with DD showed reduced pupillary responses on trials involving distractor rejection and missed targets. These physiological patterns correlated primarily with individual cognitive scores rather than acoustic salience. Conclusions. Taken together, these findings point toward an important role of individually varying cognitive resources during phonological processing and highlight the value of pupillometry as a sensitive, real-time index of cognitive engagement during a phonological task. Full article