We have analyzed the loss of agricultural land in a case study of the community Eggelsberg in the Innviertel in Upper Austria. The trigger for the project was the attribution of Austria as Europe’s ...leader in soil sealing. We compared the results of the Austrian agricultural soil survey of the 1970s with the actual land use and quantified the land use change. Within half a century, 15% of the agricultural area was converted to settlement area or to other forms of infrastructure. According to the original soil assessment, 96 ha of high-quality cropland, 1,220 ha of medium-quality cropland, 121 ha of low-quality cropland, and 409 ha of grassland were converted. The forest cover remained stable. The highest share of soil sealing was owed to the expansion of the settlement area and the construction of a successful enterprise. Minor contributions were expansions of farm buildings. The loss of agricultural land was reasonable and reflected the needs of the local population. A special protection status because of the future relevance of the agricultural land was not communicated to land managers and is not reflected in the market prices of agricultural products. The relevance of the agricultural land beyond the regional level is not fully recognized.
•We provide comprehensive data of SOC for managed land use in Austria.•SOC densities are highest in peatlands forests, and partially in grasslands.•Cropland soils are already managed to conserve the ...SOC stock.•The data serve as reference for the effect of future land management concepts.•Efforts to increase the SOC stocks are hampered by soil sealing.
We compiled information from different sources in order to establish a comprehensive map of the stock of soil organic carbon (SOC) in the upper 30 cm under different forms of land use for Austria. The information serves as a baseline for the evaluation of the potential of climate-change mitigation measures. SOC sequestration plays an important role in the discussion of terrestrial carbon (C) sinks and the size of the SOC pool is one of several quality measures for crop production and the national and regional food security. The baseline serves also for the evaluation of the effectiveness of adaptive land management in order to cope with climate change. Austrian croplands, grasslands, forests, and settlements contain 300 Mt SOC. Peatlands have the highest SOC density (220 t C/ha), yet cover only about 2% of the country. Forest soils store 106 t C/ha and comprise the largest pool due to the coverage of more than 4 Mha (48% of the country). Intensively and extensively managed grasslands cover 0.8 Mha (10%) and contain between 91 and 113 t C/ha, and cropland on 1.28 Mha (15%) hold on average 62 t C/ha. Due to the geographic heterogeneity of Austria with respect to climatic conditions, geology and soils, and topography the regional differences in SOC stocks are large. Measures to increase the SOC stock in cropland have been applied for 25 years within agri-environmental and climate-smart strategies. An increase of the total SOC pool is expected due to the afforestation and reforestation of marginal agricultural land and to a smaller extent due to the restoration of peatlands. A decline of the SOC stock is a consequence of land development for settlements and infrastructure.
Spinal muscular atrophy (SMA) is a genetic neuromuscular disease caused by mutations of the SMN1 gene. Deficient SMN protein causes irreversible degeneration of alpha motor neurons characterized by ...progressive muscle weakness and atrophy. Considering that SMA is a multi-systemic disorder and SMN protein was found to be expressed in cortical structures, the cognitive profile of adult patients with SMA has recently been of particular interest. With nusinersen, a novel, disease-modifying drug has been established, but its effects on neuropsychological functions have not been validated yet. Aim of this study was to investigate the cognitive profile of adult patients with SMA during treatment initiation with nusinersen and to reveal improvement or deterioration in cognitive performance.
This monocentric longitudinal study included 23 patients with SMA type 2 and 3. All patients were assessed with the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) before and after 14 months of treatment initiation with nusinersen. Additionally, motor function was evaluated by Hammersmith Functional Motor Scale Expanded (HFMSE), Revised Upper Limb Module (RULM) and Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R).
Of the treatment-naive patients, only three were below the age- and education-matched cut-off for cognitive impairment in the ECAS total score. Significant differences between SMA type 2 and 3 were only detected in the domain of Language. After 14 months of treatment, patients showed significant improvement of absolute scores in all three ALS-specific domains, in the non-ALS-specific domain of Memory, in both subscores and in the ECAS total score. No associations were detected between cognitive and functional outcome measures.
In some adult patients with SMA abnormal cognitive performance in ALS-specific functions of the ECAS was evident. However, the presented results suggest no clinically significant cognitive changes during the observed treatment period with nusinersen.
The course of amyotrophic lateral sclerosis (ALS,) associated with progressive physical limitations, is a challenge to the patients themselves and also to their family caregivers, who have to deal ...with psychosocial, socio-medical and organizational issues. Caregivers are often closely involved and heavily burdened themselves, which is why specific support is recommended. The aim of this study was to investigate the feasibility and acceptance of psychologically guided supportive group meetings for family caregivers in a specialist ALS outpatient clinic.
Over a period of two years, data were collected from a total of 26 caregivers of ALS patients in order to evaluate the relevance, usefulness and criticisms of open-topic meetings that took place every three months.
Topics discussed in the meetings included mainly psychosocial issues such as self-care, dealing with emotions or with conflicts with the patients and third parties, as well as practical and organizational matters. The meetings were predominantly rated as helpful, well understandable and personally relevant and the exchange in a "community of destiny" was perceived as emotionally relieving.
The ALS caregiver group meetings in the described format were easy to carry out and well accepted. Supportive interventions, such as the one reported here, might be a valuable component of ALS care, to relieve the highly burdened caregivers of ALS-patients by providing them with social, emotional and practical support. However, the quantitative verification of the intervention's effectiveness is challenging - both methodologically and due to the caregivers' complex life situation. Psychosocial support services for ALS caregivers are feasible with little effort and should be an integral part of the standard ALS care based on a multi-dimensional, palliative care concept.
: The palmomental reflex (PMR) is commonly interpreted as a frontal release sign, but it has also been discussed as a clinical marker of motoneuron affection in amyotrophic lateral sclerosis (ALS). ...The aim of this study was to investigate the impact of motor dysfunction versus neurocognitive impairment on the appearance of PMR in amyotrophic lateral sclerosis (ALS).
: 97 patients with ALS and ALS-variants were enrolled in this prospective, cross-sectional study. PMR was examined in a standardized procedure and the neurocognitive profile was assessed using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS). Disease severity and motor function were recorded using ALS Functional Rating Scale revised (ALSFRS-R) and standardized clinical assessment.
52% of all patients had a positive PMR (PMR+). These patients showed more frequently signs of motor dysfunction in the bulbar region (
< 0.001), impaired cognitive performance in the ECAS ALS-specific score (
< 0.05), predominantly in executive functions (
< 0.01), as well as lower scores in ALSFRS-R (
< 0.05) compared to patients without PMR (PMR-). A multivariate logistic regression analysis revealed that bulbar involvement, executive function impairment, and a lower motor and respiratory (non-bulbar) ALSFRS-R significantly predicted PMR+ (all
< 0.05), with bulbar involvement being a stronger predictor than executive function impairment.
In this study, we showed that bulbar involvement is a much stronger predictor on the appearance of PMR compared to executive function impairment. PMR is therefore primarily a sign for bulbar involvement, rather than a sign for executive dysfunction in ALS patients.
Multidimensional socio-medical care with an early integration of palliative principles is strongly recommended in amyotrophic lateral sclerosis (ALS), but provided inconsistently. We conducted ...telephone interviews with 49 former caregivers of deceased ALS patients to examine their experience of care in the terminal phase including caregiver burden. Patients who received specialized palliative care (45% of patients) were more likely to die at home (
= 0.004) and without burdening symptoms (
= 0.021). The majority of caregivers (86%) reported deficits in socio-medical care. Most frequently mentioned were problems receiving medical aids (45%) and a lack of caregiver support (35%). A higher level of deficits experienced by caregivers was associated with negative health outcomes on the side of the caregivers (reported by 57% of them;
= 0.002) and stronger caregiver burden (
= 0.004). To provide good quality of dying to patients and reduce the burden on caregivers, multidimensional-including palliative-care in ALS urgently needs to be strengthened in the healthcare structures.
Amyotrophic lateral sclerosis (ALS) can result into an incomplete locked in state (iLIS), in which communication depends on eye tracking computer devices. Oculomotor function impairments in ALS have ...been reported, but there is little research, particularly with respect to patients in iLIS. In the present study, we compared reflexive and executive oculomotor function by means of an eye tracking test battery between three groups: advanced ALS patients in iLIS (n = 22), patients in early to middle ALS stages (n = 44) and healthy subjects (n = 32). Patients with ALS showed significant deteriorations in oculomotor functions, with stronger impairments in iLIS. More specifically, ALS patients produced visually guided prosaccades with longer latencies and more frequent hypometria compared to healthy subjects. Longest latencies were obtained in iLIS patients, with a stronger prolongation for vertical than for horizontal prosaccades. ALS patients made more antisaccade errors and generated antisaccades with longer latencies. Smooth pursuit was also impaired in ALS. In the earlier ALS stages, bulbar onset patients presented stronger antisaccade and smooth pursuit deficits than spinal onset patients. Our findings reveal a relevant deterioration of important oculomotor functions in ALS, which increases in iLIS. It includes impairments of reflexive eye movements to loss of executive inhibitory control, indicating a progressing pathological involvement of prefrontal, midbrain and brainstem areas. The assessment of oculomotor functions may therefore provide clinically relevant bio- and progression marker, particularly in advanced ALS.
For both patients with amyotrophic lateral sclerosis (ALS) and their next of kin (NOK), the maintenance of quality of life (QoL) and mental health is particularly important. First studies suggest ...significant discrepancies between QoL reports by patients and NOK, but little is known for advanced ALS stages. To address this issue, we screened 52 ALS patients in incomplete locked-in state (iLIS). Final results were obtained for 15 couples of iLIS patients and NOK. We assessed patients’ and NOK’s subjective QoL, depression and anxiety and NOK’s caregiver burden. Gaze controlled questionnaires allowed direct assessment of patients. Patients and NOK self-reported comparable, mostly moderate to high levels of QoL. Of note, NOK indicated stronger anxiety symptoms. Higher anxiety levels in NOK were associated with stronger caregiver burden and reduced QoL. No significant misjudgment of patient’s QoL by the NOK was evident, while patients overestimated NOK’s global QoL. However, NOK with severe caregiver burden and depression symptoms gave poorer estimations of patients’ QoL. This relationship is relevant, considering NOK’s impact on life critical treatment decisions. While the daily time NOK and patient spend together was positively correlated with NOK’s QoL and mental health, this was not reversely found for the patients. Our results suggest that NOK adapt less successfully to the disease and concomitant experience of loss and point to an urgent need for specialized psychosocial support. The findings emphasize the importance of direct psychological wellbeing assessment of both patients and NOK in clinical practice, enabled by eye-tracking technology for patients in iLIS.
Objective
To determine whether serum creatine kinase activity (CK) and serum creatinine concentration (Crn) are prognostic and predictive biomarkers for disease severity, disease progression, and ...nusinersen treatment effects in adult patients with 5q‐associated spinal muscular atrophy (SMA).
Methods
Within this retrospective, multicenter observational study in 206 adult patients with SMA, we determined clinical subtypes (SMA types, ambulatory ability) and repeatedly measured CK and Crn and examined disease severity scores (Hammersmith Functional Motor Scale Expanded, Revised Upper Limb Module, and revised Amyotrophic Lateral Sclerosis Functional Rating Scale). Patients were followed under nusinersen treatment for 18 months.
Results
CK and Crn differed between clinical subtypes and correlated strongly with disease severity scores (e.g., for Hammersmith Functional Motor Scale Expanded: (CK) ρ = 0.786/ (Crn) ρ = 0.558). During the 18 months of nusinersen treatment, CK decreased (∆CK = −17.56%, p < 0.0001), whereas Crn slightly increased (∆Crn = +4.75%, p < 0.05).
Interpretation
Serum creatine kinase activity and serum creatinine concentration reflect disease severity of spinal muscular atrophy and are promising biomarkers to assess patients with spinal muscular atrophy during disease course and to predict treatment response. The decrease of creatine kinase activity, combined with the tendency of creatinine concentration to increase during nusinersen treatment, suggests reduced muscle mass wasting with improved muscle energy metabolism.
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