Lance-Adams syndrome (LAS) is a rare condition described as chronic intentional myoclonus following prolonged hypoxia. With limited cases of the condition, no standardised diagnostic criteria, and features overlapping acute myoclonus status epilepticus (MSE), diagnosis and management are challenging.Our case is a man in his 20s, who was admitted to the intensive care unit following two in-hospital cardiac arrests. He consequently developed persistent generalised myoclonic seizures despite multiple antiepileptics and sedation. CT, MRI and electroencephalogram (EEG) ruled out a diagnosis of MSE, making LAS more likely. Ketamine was ultimately required to terminate the generalised myoclonic seizures after which antiepileptic drugs were optimised to reduce residual intention myoclonus.This report highlights key features prompting the clinical suspicion of LAS in a patient who has no significant evidence of brain injury, preserved consciousness with continued severe generalised myoclonus and outlines pharmacological and neurophysiological management.

Although the initial presenting cardiac rhythm is an established prognostic factor for extracorporeal cardiopulmonary resuscitation, heart rhythm dynamics during conventional cardiopulmonary resuscitation remain poorly studied. We evaluated how the evolution of rhythm affects neurological outcomes and survival among recipients of extracorporeal cardiopulmonary resuscitation.

We systematically searched PubMed and Embase for studies evaluating rhythm dynamics, focusing on Rhythm Improvement (from non-shockable to shockable) or Rhythm Decay (from shockable to non-shockable). The primary outcome was survival with a favorable neurological outcome at the longest follow-up; the secondary outcome was overall survival. A random-effects model analysis with the Hartung-Knapp adjustment was used.

Ten observational studies evaluating 5183 subjects with out-of-hospital cardiac arrest treated with extracorporeal cardiopulmonary resuscitation were included. Rhythm improvement was associated with a higher probability of a favorable neurological outcome (RR 3.12, 95% CI 1.26-7.74; GRADE: Low) compared with refractory non-shockable rhythms. Conversely, rhythm decay reduced the likelihood of a favorable neurological outcome compared with a sustained shockable rhythm (RR 0.41, 95% CI 0.21-0.80; GRADE: Moderate). Notably, decay toward asystole showed a robust negative association with good neurological recovery (RR 0.20, 95% CI 0.09-0.45; GRADE: Moderate), whereas decay toward pulseless electrical activity did not reach statistical significance (RR 0.62, 95% CI 0.31-1.25; GRADE: Low).

Rhythm dynamics provide valuable prognostic insights for candidates for extracorporeal cardiopulmonary resuscitation. Rhythm decay toward asystole may predict resuscitation failure and poor neurological survival, suggesting that it should prompt a critical re-evaluation of the appropriateness of extracorporeal cardiopulmonary resuscitation.

Bi-allelic variants in IDUA classically cause mucopolysaccharidosis type I (MPS I), a multisystem lysosomal storage disorder. This study describes 14 individuals from 12 families presenting with retinitis pigmentosa, later attributed to bi-allelic IDUA genotypes. Notably, seven individuals, despite comprehensive systemic assessments, showed no features consistent with MPS I at last assessment (aged 46-73 years). Leukocyte α-L-iduronidase activity was reduced but not abolished across all individuals assessed (n = 9), supporting a shared mechanistic basis for the mild, retina-predominant, or exclusively retinal phenotype. No individual carried bi-allelic null genotypes; most had a loss-of-function allele paired with a missense variant, consistent with an overall hypomorphic genotype. Variant-level functional assays, using a cell-based expression platform assessing enzyme activity and protein abundance to derive relative specific activity (RSA), showed that several missense alleles confer very low residual activity and/or impaired abundance/processing, with RSA typically in the ∼0.5%-1.5% range. The recurrent missense c.298A>G (p.Arg100Gly) variant showed no protein or catalytic effect; targeted long-read transcript analysis instead demonstrated predominant loss-of-function mis-splicing with a small fraction of correctly spliced transcript (∼1%-2%), explaining residual activity in vivo. Together, these data expand the phenotypic spectrum of IDUA-associated disease to include late-onset retinal-predominant or apparently isolated retinitis pigmentosa and indicate that residual enzyme activity likely contributes to systemic sparing, highlighting the value of integrated genomic, biochemical, and functional analyses to advance understanding in attenuated metabolic disease.

Because populations in many countries are aging, a number of large database studies have reported more patients with Parkinson disease undergoing TKA, and the risk of complications after surgery is greater in this population. Because Parkinson disease-related autonomic dysfunction, medication changes, mobility loss, and frailty may evolve after diagnosis, the diagnosis-to-TKA interval could identify clinically different vascular and periprosthetic risk profiles and help guide counseling and selective preoperative assessment; however, 5-year endpoint-specific risks remain insufficiently characterized.

(1) Is Parkinson disease diagnosed before TKA associated with higher 5-year risks of vascular events and periprosthetic complications after TKA? (2) How do these associations appear when patients with Parkinson disease diagnosed before TKA are grouped by a pragmatic 5-year diagnosis-to-TKA interval?

Using the National Health Insurance Service (NHIS) database of South Korea, we identified patients who underwent unilateral TKA between January 2010 and December 2018, with follow-up available through December 2023 to allow assessment of 5-year postoperative outcomes. The database links procedure, diagnosis, prescription, and health-screening data at the individual level, allowing assessment of 5-year vascular and periprosthetic outcomes. Patients with < 5 years of follow-up or preexisting cardiovascular or cerebrovascular disease were excluded to focus on incident postoperative vascular events. The unmatched analytic cohort included 287,075 patients without Parkinson disease and 20,728 patients with Parkinson disease diagnosed before TKA, including 16,877 diagnosed ≤ 5 years before TKA and 3851 diagnosed > 5 years before TKA. All patients in these unmatched groups had at least 5 years of follow-up by design. Parkinson disease was defined by diagnostic codes and prescriptions for Parkinson disease-related medications. Each group of patients diagnosed with Parkinson disease before TKA was propensity score-matched 1:1 to the group of patients without Parkinson disease, yielding 20,707 matched pairs for all patients with Parkinson disease before TKA, 16,860 matched pairs for those diagnosed ≤ 5 years before TKA, and 3848 matched pairs for those diagnosed > 5 years before TKA. After matching, age, sex, comorbidity, health behaviors, dyslipidemia, and year of surgery were balanced. Outcomes were analyzed using Cox regression models, with prespecified time-window analyses to describe temporal patterns in adjusted associations.

After adjustment for age, sex, Charlson comorbidity index, smoking, alcohol use, physical activity, dyslipidemia, and year of surgery, Parkinson disease diagnosed before TKA was associated with a slightly higher 5-year adjusted hazard of cerebrovascular events (adjusted HR 1.11 [95% confidence interval (CI) 1.07 to 1.16]; p < 0.001) but not with cardiovascular events, periprosthetic fracture, or PJI. Among patients with Parkinson disease diagnosed ≤ 5 years before TKA, cardiovascular events (adjusted HR 1.05 [95% CI 1.01 to 1.10]; p = 0.02) and cerebrovascular events (adjusted HR 1.14 [95% CI 1.09 to 1.18]; p < 0.001) were associated with slightly higher adjusted hazards than in matched patients without Parkinson disease. These associations were not observed in patients with Parkinson disease diagnosed > 5 years before TKA. Time-window analyses in patients diagnosed ≤ 5 years before TKA showed a small early inverse association for cardiovascular events at 6 months and a small positive association by 5 years. Periprosthetic fracture and periprosthetic joint infection were not increased across interval subgroups.

These findings should not be interpreted as supporting routine cardiovascular or cerebrovascular screening solely because a patient has Parkinson disease. Rather, Parkinson disease status and the diagnosis-to-TKA interval may help surgeons counsel patients and identify selected individuals who warrant multidisciplinary perioperative assessment of vascular risk factors, autonomic symptoms, blood pressure instability, and Parkinson disease medication management. Future studies should incorporate clinical Parkinson disease severity, frailty, mobility, and competing mortality to improve absolute risk prediction and identify patients who may benefit from additional preoperative medical review.

Level III, therapeutic study.

G protein-coupled receptor (GPCR) signalling plays a key role in the regulation of cardiovascular function, and in many cases, dysregulation of these events is a critical driver of disease progression. Drugs modulating GPCRs including β-blockers and angiotensin receptor blockers (ARBs) have served as largely symptomatic treatments spanning across several cardiovascular diseases. However, with few exceptions, most patients are treated with the same drugs in the same order regardless of etiology, often affecting disease progression. Thus, moving towards more personalized approaches to treating patients might improve outcomes. Over the past two decades, advances in induced pluripotent stem cell (iPSC)-derived cells have been transformative in accelerating our understanding of cardiovascular cell signalling in a patient-specific manner. In this review, we focus on how iPSC-derived cardiac models have increased our understanding of GPCR signalling in the context of cardiovascular disease, how these models have been used to investigate responses to clinically used drugs, and how these approaches might translate to the development of more personalized approaches and novel GPCR-targeting drugs.

Emerging evidence highlights the contribution of systemic inflammation and oxidative stress to the pathogenesis and progression of Parkinson's disease (PD). Accordingly, blood-based biomarkers that capture these processes are of increasing clinical interest. This study evaluated the association between PD and serum uric acid, serum albumin, and their integrated index-the uric acid-to-albumin ratio (UAR)-to determine their potential utility as biomarkers of systemic inflammation, oxidative stress, and disease progression.

A total of 153 patients with PD and 291 healthy controls were included. Serum uric acid, albumin, and UAR were measured through standardized biochemical assays. Group comparisons and multivariable logistic regression were performed to assess associations with PD, and analyses stratified by Hoehn and Yahr stage examined relationships with disease severity.

Uric acid, albumin, and UAR levels were significantly lower in patients with PD compared with healthy controls (all p < 0.001). After adjustment for age, sex, hypertension, and diabetes, UAR remained independently associated with PD, with lower UAR corresponding to higher odds of disease (OR = 0.72, 95% CI: 0.63-0.82, p < 0.001). Stratified analyses showed that UAR reductions were most prominent in early and middle PD stages, whereas advanced-stage values approximated those of controls. ROC analysis demonstrated moderate discriminative ability of UAR for PD overall (AUC = 0.630) and for early-to-middle stage disease (AUC = 0.620 and 0.644, respectively).

UAR is significantly reduced in PD and associated with early-middle disease stages, supporting its potential as an accessible biomarker for PD monitoring, though larger longitudinal studies are needed for validation.

Postpartum depression is a disorder that disrupts maternal functioning and compromises infant well-being. This study determined the prevalence of postpartum depression and investigated associated factors among mothers attending health centers in Abomey.

&amp;#160;This was a cross-sectional analytical study involving 440 mothers with infants aged three months or younger attending health centers in Abomey. Following random selection of health centers, all eligible mothers were surveyed using a questionnaire. The Edinburgh Postnatal Depression Scale, with a cut-off score of 13, was used to screen for postpartum depression. Logistic regression modelling was used to explore associated factors. The strength of the association was assessed using odds ratios and their 95% confidence intervals (CI).

The prevalence of postpartum depression was 17.05% (95% CI: 13.80%&amp;#8211;20.87%). Factors associated with postpartum depression included marital status, low maternal decision-making power within the household, physical violence by a partner, unwanted pregnancy, and stigma or discrimination experienced during childbirth.

There is evidence to suggest that mothers may suffer from depression after childbirth. These findings may guide future research aimed at better understanding this phenomenon, as well as initiatives to improve maternal mental health in Benin.

Gambling is growing at an alarming rate in Benin, particularly among students. This population, seeking independence and success, is especially vulnerable to the risks of disorders associated with these practices.

to determine the prevalence of, and identify factors associated with disorders related to gambling in universities in Benin.

This is a cross-sectional analytical study involving 1,360 Beninese students. Gambling-related disorders were assessed using the diagnostic criteria of the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-V). Associated factors were explored using multiple logistic regression.

Of the 1,360 students, 455 (33.5%) were gamblers, 89 of whom were classified as having gambling-related disorders, representing a prevalence of 6.5% among all students and 19.6% [95% CI: 15.9%&amp;#8211;23.2%] among gamblers. The distribution of disorders was as follows: 12.1% mild, 6.8% moderate, and 0.7% severe. After adjustment, factors associated with these disorders were age (p=0.007), having a gambling app on one&amp;#8217;s phone (p&amp;lt;0.001), friends&amp;#8217; influence on decision-making (p=0.014), frequency of gambling (p=0.012), betting with money belonging to others (p=0.006), duration of engagement in online gambling (p=0.032), and anxiety (p&amp;lt;0.001).

This study highlights the extent of gambling-related disorders in Beninese universities. It emphasizes the need for stricter regulation and targeted preventive measures to address this still insufficiently recognized public health problem.

Persons with substance use disorders (SUDs) have markedly increased risk for lifestyle-related diseases and reduced life expectancy. Initial findings suggest that high-intensity physical training (HIT) represents an efficient and effective training modality for improving physical and functional capacity for persons with SUDs. However, acheiving such improvements depends not only on the availability of such training modalities but also on adherence, which remains insufficiently understood in this population. Thus, to extend the benefits of HIT to additional persons within this population, there is a need for a broader, deeper, and more patient-centred understanding of their experiences with HIT integrated into specialised treatment, including facilitators and barriers to adherence.

This protocol article presents an ongoing qualitative collaborative health service study where inpatients with SUDs are interviewed in-depth about their experiences of and adherence to HIT by semi-structured interview guides. Approximately 20 inpatients with SUDs will be purposively recruited based on relevant background variables and inclusion criteria to ensure breadth in experiences: 10 who have adhered to supervised HIT as part of specialised SUD treatment and 10 who have not. Depth will be supported through in-depth interviewing, reflexive field notes, and collaborative thematic analysis with persons with lived experiences.

The study has received ethical approval from Regional Committees for Medical and Health Research Ethics (Reference ID: 2019/501). By strengthening the knowledge base on factors influencing adherence and non-adherence in HIT as part of clinical practice, the findings may support patient's adherence to HIT integrated in SUD treatment in a more person-centred way, ultimately benefiting the physical and functional capacity for additional persons with SUDs. Findings will be disseminated together with persons with lived experiences via conferences, peer-reviewed publications, as well as popular-scientific communication and media. Finally, the findings may generate theoretically informative insights into patients' experiences and mechanisms influencing adherence to HIT, as well as novel concepts and insights needed for new research directions within an increasingly prioritised field in specialised SUD treatment.