BackgroundMultiple system atrophy (MSA) is a fatal and still poorly understood degenerative movement disorder that is characterised by autonomic failure, cerebellar ataxia, and parkinsonism in various combinations. Here we present the final analysis of a prospective multicentre study by the European MSA Study Group to investigate the natural history of MSA.MethodsPatients with a clinical diagnosis of MSA were recruited and followed up clinically for 2 years. Vital status was ascertained 2 years after study completion. Disease progression was assessed using the unified MSA rating scale (UMSARS), a disease-specific questionnaire that enables the semiquantitative rating of autonomic and motor impairment in patients with MSA. Additional rating methods were applied to grade global disease severity, autonomic symptoms, and quality of life. Survival was calculated using a Kaplan-Meier analysis and predictors were identified in a Cox regression model. Group differences were analysed by parametric tests and non-parametric tests as appropriate. Sample size estimates were calculated using a paired two-group t test.Findings141 patients with moderately severe disease fulfilled the consensus criteria for MSA. Mean age at symptom onset was 56·2 (SD 8·4) years. Median survival from symptom onset as determined by Kaplan-Meier analysis was 9·8 years (95% CI 8·1–11·4). The parkinsonian variant of MSA (hazard ratio [HR] 2·08, 95% CI 1·09–3·97; p=0·026) and incomplete bladder emptying (HR 2·10, 1·02–4·30; p=0·044) predicted shorter survival. 24-month progression rates of UMSARS activities of daily living, motor examination, and total scores were 49% (9·4 [SD 5·9]), 74% (12·9 [8·5]), and 57% (21·9 [11·9]), respectively, relative to baseline scores. Autonomic symptom scores progressed throughout the follow-up. Shorter symptom duration at baseline (OR 0·68, 0·5–0·9; p=0·006) and absent levodopa response (OR 3·4, 1·1–10·2; p=0·03) predicted rapid UMSARS progression. Sample size estimation showed that an interventional trial with 258 patients (129 per group) would be able to detect a 30% effect size in 1-year UMSARS motor examination decline rates at 80% power.InterpretationOur prospective dataset provides new insights into the evolution of MSA based on a follow-up period that exceeds that of previous studies. It also represents a useful resource for patient counselling and planning of multicentre trials.FundingFifth Framework Programme of the European Union, the Oesterreichische Nationalbank, and the Austrian Science Fund.
La energia hidroelectrica esta siendo fomentada a nivel mundial por ser renovable y limpia. Sin embargo, su produccion conlleva impactos al ecosistema fluvial, destacando las consecuencias ecologicas de la alteracion de los regimenes naturales de caudales. Concretamente, las centrales a pie de presa operan de forma intermitente de acuerdo a los precios y a la demanda de energia, que varian de forma instantanea, provocando el fenomeno del hydropeaking. El hydropeaking consiste en la variacion frecuente y rapida del caudal en un breve periodo de tiempo, generalmente a lo largo del dia (variaciones intra-dia o instantaneas), y que resulta en alteraciones de parametros hidraulicos como el nivel del agua, la velocidad del flujo, o la tension de arrastre, de la morfologia fluvial y la calidad del agua. En este trabajo se presenta una herramienta informatica (InSTHAn) para el analisis de series largas de caudal y nivel medidas a cualquier escala temporal intra-diaria. InSTHAn permite caracterizar y evaluar el grado de alteracion de una serie instantanea de caudales (y niveles) mediante el calculo rapido y sencillo de 35 indicadores (Short-Term Characterization e Impact Indicators; STCI y STII). Tambien se discute la utilidad de InSTHAn en el desarrollo de modelos hydropeaking ? impacto ecologico. Para ello, se presentan de forma sucinta los resultados de un ensayo experimental en el que se relaciono el hydropeaking con el estado de las comunidades riparias. Ume y Vindel han sido los rios escogidos en el norte de Suecia para la aplicacion de InSTHAn y los ensayos de campo, el primero regulado por una serie de centrales hidroelectricas, y el segundo totalmente inalterado. El conocimiento cientifico y las nuevas herramientas generados en este trabajo permiten identificar los escenarios de operacion que minimizan el impacto sobre el bosque ripario y maximizan la produccion hidroelectrica, asi como las mas efectivas estrategias de restauracion de dicho ecosistema.
Recent research has emphasized the impor- tance of riparian ecosystems as centers of biodiversity and links between terrestrial and aquatic systems. Riparian eco- systems also belong among the environments that are most disturbed by humans and are in need of restoration to main- tain biodiversity and ecological integrity. To facilitate the com- pletion of this task, researchers have an important function to communicate their knowledge to policy-makers and manag- ers. This article presents some fundamental qualities of ripar- ian systems, articulated as three basic principles. The basic
Abstract Insulin‐like growth factor II (IGF‐II) is expressed and secreted by the choroid plexus and has been suggested to act as a trophic factor in the adult mammalian central nervous system. The aim of the present study was to investigate whether IGF‐II has an autocrine role in the choroid plexus. Using in situ hybridization we demonstrate that IGF‐II is primarily expressed in the epithelium of adult rat choroid plexus. Conditioned medium from primary cultures of purified rat choroid plexus epithelial cells, intact choroid plexus tissue, as well as rat CSF, displaced IGF‐II binding to a 23 HMM melanoma cell line in an IGF‐II radioreceptor assay. The presence of IGF‐II and IGF binding protein‐2 in conditioned medium was shown by Western immunoblot. The mitotic activity in choroid plexus epithelial cell cultures was quantified by immunohistochemical staining of bromodeoxyuridine incorporated into cell nuclei. A monoclonal antibody towards IGF‐II inhibited cell division by 35%, while IGF‐I increased the number of stained nuclei by 75%. Basic fibroblast growth factor stimulated cell division at low concentrations, but had no effect at high concentrations. Growth hormone had no effect. We conclude that IGF‐II in the choroid plexus could have an autocrine role in the regulation of choroid plexus epithelial cell growth.
Results of multiple comparison tests in cases when repeated-measures generalized or general linear models indicated a significant reach type or treatment effect.
Abstract Ecological restoration is becoming an increasingly common management tool worldwide. However, a challenge still exists on how to effectively monitor restoration outcomes and evaluate restoration success for ecological restoration managers. In this review, the goal is to evaluate whether the research in a degraded area has been sufficient for fostering efficient restoration measures and follow‐up of restoration success based on the Society for Ecological Restoration (SER) criteria. We selected the Inner Mongolian Steppe (IMS) in China as a model system. This area has been the subject of substantial research over the most recent years to understand degradation processes and restoration outcomes. We put together the variables used to assess degradation and restoration needs in the IMS and analyzed restoration results based on SER's nine criteria for evaluating restoration success. We found that the accomplished research in the IMS only partially supplied the data needed for evaluation of restoration success. The available results were sufficient for a proper evaluation of species composition and tentatively supported assessments of another seven criteria but not self‐sustainability. Grazing exclusion led to the fastest and most successful recovery of degraded steppe, but landscape‐scale processes during restoration in the IMS are still incompletely known. Our review supports large‐scale restoration of the IMS and emphasizes the need for long‐time monitoring for a more complete evaluation of the outcome of the IMS restoration following all SER's criteria.
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