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Twenty-five years since foundational publications on valuing ecosystem services for human well-being1,2, addressing the global biodiversity crisis3 still implies confronting barriers to incorporating nature’s diverse values into decision-making. These barriers include powerful interests supported by current norms and legal rules such as property rights, which determine whose values and which values of nature are acted on. A better understanding of how and why nature is (under)valued is more urgent than ever4. Notwithstanding agreements to incorporate nature’s values into actions, including the Kunming-Montreal Global Biodiversity Framework (GBF)5 and the UN Sustainable Development Goals6, predominant environmental and development policies still prioritize a subset of values, particularly those linked to markets, and ignore other ways people relate to and benefit from nature7. Arguably, a ‘values crisis’ underpins the intertwined crises of biodiversity loss and climate change8, pandemic emergence9 and socio-environmental injustices10. On the basis of more than 50,000 scientific publications, policy documents and Indigenous and local knowledge sources, the Intergovernmental Platform on Biodiversity and Ecosystem Services (IPBES) assessed knowledge on nature’s diverse values and valuation methods to gain insights into their role in policymaking and fuller integration into decisions7,11. Applying this evidence, combinations of values-centred approaches are proposed to improve valuation and address barriers to uptake, ultimately leveraging transformative changes towards more just (that is, fair treatment of people and nature, including inter- and intragenerational equity) and sustainable futures. © 2023, The Author(s).

Background Fatigue is common in children and adolescents with multiple sclerosis (MS) and its aetiology is assumed to be multifactorial, however, its relationship to sleep quality in this population remains unknown. This study aims to examine the prevalence of fatigue and sleep disturbance in this population and their relationship to mood, quality of life, physical activity, and MS disease characteristics. Methods Children with pediatric onset multiple sclerosis (POMS) aged 0–18 were recruited. Subjective sleep quality was assessed by the Pediatric Daytime Sleepiness Scale (PDSS), Sleep Disturbance Scale for Children (SDSC) and OSA-18. All children were referred for polysomnography (PSG) including transcutaneous CO2 and video monitoring. Fatigue was assessed using the PedsQL Multidimensional Fatigue Scale (PedsQL-MFS). Progress to date: Fifteen children with relapsing remitting MS (mean age 15.73±1.44, mean EDSS score 1.11±1.12) have been enrolled to date. 73% of children were fatigued according to the PedsQL-MFS (mean transformed score 52.04). 67% and 60% of children scored higher than the clinical cutoff for the PDSS (17.87) and SDSC (42.73) respectively. However, all children scored within the normal range for the OSA-18 (34.00). To date, eleven children have completed PSG. Intended outcome and impact: This is the first study utilising PSG to objectively assess sleep quality in children with POMS. Findings from this study will document the magnitude of sleep disturbance in this population and have implications for the management of fatigue and other related impairments observed in paediatric MS.

Objective: This study aims to determine valid and responsive gait and balance outcome measures to detect progressive change over 12 months for individuals with FRDA. Background(s): Gait ataxia and instability are common presenting features of Friedreich ataxia (FRDA). Mobility declines with disease progression until ambulation is no longer possible, approximately 10-15 years following initial symptoms. Design/Methods: Fifty-one individuals with FRDA underwent assessment at baseline and six months (12-month data is being collected). Measures included (i) gait parameters at preferred and fast speeds using the GAITRite instrumented walkway, (ii) Biodex Balance SystemTM postural stability test (PST) and limits of stability (LOS), (iii) Berg Balance Scale (BBS), (iv) Timed 25 Foot Walk Test, (v) Dynamic Gait Index and (iv) Friedreich Ataxia Rating Scale (FARS) upright stability subscale. Correlations between objective measures, the FARS neurological exam, Scale for the Assessment and Rating of Ataxia (SARA) and disease characteristics were examined. The standardised response mean was reported as the effect size index (ES) for comparison of internal responsiveness. Result(s): Significant correlations were found between the BBS and SARA (p<0.001). Increased stride time variability was associated with a higher FARS score (p<0.001). The SARA and FARS did not detect a significant change over six months. However, the FARS upright stability subscale significantly increased with an ES of 0.571 (p<0.001) over this time. The BBS (ES: -0.519, p=0.001) and PST anteriorposterior index with eyes-closed (ES=0.667, p=0.010) also detected balance decline. A significant decrease in stride length during fast walking was also evident (ES=-0.323, p=0.030). Conclusion(s): Clinical balance measures, PST eyes-closed anterior-posterior index and stride length are more sensitive to the decline in individuals with FRDA as compared to previously validated measures of disease severity, the SARA and

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy with a highly variable clinical presentation, course, and outcome. The factors that determine the clinical variation of GBS are poorly understood which complicates the care and treatment of individual patients. The protocol of the ongoing International GBS Outcome Study (IGOS), a prospective, observational, multicenter cohort study that aims to identify the clinical and biological determinants and predictors of disease onset, subtype, course and outcome of GBS is presented here. Patients fulfilling the diagnostic criteria for GBS, regardless of age, disease severity, variant forms, or treatment, can participate if included within 2 weeks after onset of weakness. Information about demography, preceding infections, clinical features, diagnostic findings, treatment, course, and outcome is collected. In addition, cerebrospinal fluid and serial blood samples for serum and DNA is collected at standard time points. The original aim was to include at least 1,000 patients with a follow-up of 1–3 years. Data are collected via a web-based data entry system and stored anonymously. IGOS started in May 2012 and by January 2017 included more than 1,400 participants from 143 active centers in 19 countries across 5 continents. The IGOS data/biobank is available for research projects conducted by expertise groups focusing on specific topics including epidemiology, diagnostic criteria, clinimetrics, electrophysiology, antecedent events, antibodies, genetics, prognostic modeling, treatment effects, and long-term outcome of GBS. The IGOS will help to standardize the international collection of data and biosamples for future research of GBS.

Lower limb spasticity compromises the independence of people with Friedreich's ataxia (FRDA). This study sought to examine lower limb spasticity in FRDA in order to offer new insight as to the best approach and timing of spasticity management. Gastrocnemius and soleus spasticity and muscle length were measured by the Modified Tardieu Scale (MTS) in 31 participants with typical and late-onset FRDA. Relationships between the MTS and the Friedreich Ataxia Rating Scale (FARS), Functional Independence Measure (FIM), and disease duration were analysed. Differences between ambulant (n = 18) and non-ambulant (n = 13) participants were also examined. All participants had spasticity in at least one muscle, and 38.9% of ambulant and 69.2% of non-ambulant participants had contracture in one or both of their gastrocnemius muscles. Significant negative correlations were found between both gastrocnemius and soleus angle of catch and the FARS score. The FIM score also demonstrated significant correlations with gastrocnemius muscle length and angle of catch. Gastrocnemius and soleus spasticity and contracture is apparent in people with FRDA. Spasticity is evident early in the disease and in ambulant participants. Management of spasticity and reduced muscle length should be considered in people with FRDA at disease onset to optimise function.Copyright © 2016 Elsevier Ltd. All rights reserved.

info:eu-repo/semantics/published

OBJECTIVE: This is an open-label clinical trial evaluating the effect of two different doses of resveratrol on lymphocyte frataxin levels over a 12-week period in individuals with Friedreich ataxia (FRDA). Secondary aims included the effect of resveratrol on FXN mRNA, oxidative stress markers and clinical measures of disease severity. Safety and tolerability were also evaluated. BACKGROUND: FRDA is due to a triplet repeat expansion in the FXN gene, resulting in deficiency of the mitochondrial protein frataxin. Resveratrol is a plant-derived compound. It was identified to increase frataxin expression in cellular and mouse models of FRDA, and has anti-oxidant properties. DESIGN/METHODS: 27 participants were enrolled in this open-label sequential clinical pilot study. Thirteen participants received resveratrol 1g daily (low-dose), and fourteen received resveratrol 5g daily (high-dose). RESULT(S): 24 participants completed the study (twelve in each dosage group). Preliminary assessment of selected secondary outcome measures has been completed. Improvement in ataxia was evident in the high-dose group (change in ICARS, International Cooperative Ataxia Rating Scale -1.9 points, 95% CI -3.1, -0.8, p=0.004) but not the low-dose group (change in ICARS -0.3 points, 95% CI -3.2, 2.6, p=NS). A significant decrease in the oxidative stress marker plasma F2 isoprostane levels occurred in the high-dose group (-216.9 pmol/L plasma, 95% CI -301.4, -132.2, p<0.001) but not the low-dose group (-45.2 pmol/L plasma, 95% CI -152.9, 62.6, p=0.38, p=NS). Change in lymphocyte frataxin levels and other secondary outcome measures will be presented. No serious adverse events were recorded. Gastrointestinal side effects were a common dose-related adverse event. CONCLUSION(S): Preliminary analysis of results from this open-label trial suggests that treatment with high-dose resveratrol for 12 weeks improves some clinical and biological markers of FRDA. A placebo-controlled study is required to asse

Objective: Friedreich ataxia (FRDA), the most common hereditary ataxia, is due to a triplet repeat expansion in the FXN gene, resulting in deficiency of the mitochondrial protein frataxin. There is no treatment proven to alter its course. Resveratrol (Megaresveratrol, Danbury, USA) is a plant-derived compound. It was identified to increase frataxin expression in cellular and mouse models of FRDA, and is proposed to have anti-oxidant and neuroprotective properties. Design(s): Open-label clinical pilot study. Method(s): To evaluate the effect of two different doses of resveratrol on lymphocyte frataxin levels over a 12-week period. Result(s): A total of 30 participants will be enrolled (15 participants 1g resveratrol daily; 15 participants 5g resveratrol daily). The primary aim is to evaluate the effect of two doses of resveratrol on lymphocyte frataxin levels at 12weeks compared to baseline. Secondary aims will evaluate the effect of resveratrol on FXN mRNA expression, markers of oxidative stress, clinical measures of FRDA, and echocardiography findings at 12weeks compared to baseline. The safety of resveratrol and pharmacokinetic data will also be evaluated. Inclusion criteria include: (1) age >18years; (2) homozygosity for the GAA repeat expansion in the FXN gene; (3) at least minimum clinical evidence of ataxia; and (4) adequate end organ function. Enrolment for this trial commenced in May 2011. Study design and progress will be reviewed. Conclusion(s): Resveratrol shows promise as a treatment for FRDA. This clinical pilot trial will measure its effect clinically and on biomarkers over 12 weeks.