Biofragmentation of Polystyrene Microplastics: A Silent Process Performed by Chironomus sancticaroli Larvae.

Polystyrene (PS) is one of the main synthetic polymers produced around the world, and it is present in the composition of a wide variety of single-use objects. When released into the environment, these materials are degraded by environmental factors, resulting in microplastics. We investigated the ability of Chironomus sancticaroli (Diptera, Chironomidae) to promote the fragmentation of PS microspheres (24.5 ± 2.9 µm) and the toxic effects associated with exposure to this polymer. C. sancticaroli larvae were exposed to 3 different concentrations of PS (67.5, 135, and 270 particles g-1 of dry sediment) for 144 h. Significant lethality was observed only at the highest concentration. A significant reduction in PS particle size as well as evidence of deterioration on the surface of the spheres, such as grooves and cracks, was observed. In addition, changes in oxidative stress biomarkers (SOD, CAT, MDA, and GST) were also observed. This is the first study to report the ability of Chironomus sp. to promote the biofragmentation of microplastics. The information obtained demonstrates that the macroinvertebrate community can play a key role in the degradation of plastic particles present in the sediment of freshwater environments and can also be threatened by such particle pollution.

The impact of daylight-saving time (DST) on patients with delayed sleep-wake phase disorder (DSWPD).

Due to time zones, sun time and local time rarely match. The difference between local and sun time, which we designate by Solar Jet Lag (SoJL), depends on location within a time zone and can range from zero to several hours. Daylight saving time (DST) simply adds 1 h to SoJL, independently of the location. We hypothesised that the impact of DST is particularly problematic in patients with delayed sleep-wake phase disorder (DSWPD), worsening their sleep debt. DSWPD is characterised by a chronic misalignment between the internal and social timing, reflected by an inability to fall asleep and wake-up at conventional or socially acceptable times. We analysed the clinical records of 162 DSWPD patients from a sleep medicine centre in Lisbon, Portugal (GMTzone), and separated them into two groups: the ones diagnosed across DST or across Standard Time (ST). We included 82 patients (54.9% male; age: median [Q1 , Q3 ] 34.5 [25.0, 45.3]; range 16-92; 54 in DST and 28 in ST) who had Dim Light Melatonin Onset (DLMO) measured as a marker for the circadian phase and sleep timing (onset, SO, mid-point, MS and end, SE) self-reported separately for work- and work-free days. Differences between ST and DST were compared using Mann-Whitney or Student's t-tests. On a weekly average, patients in DST slept less (difference between medians of 37 min. p < .01), mainly due to sleep on workdays (SDw, p < .01), which also correlated with SoJL (rsp = .38, p < .01). While the time from DLMO to SO was similar in those in ST or those in DST, the time from DLMO to SE was significantly shorter for those in DST. The average duration between DLMO and sleep end was close to 10.5 h in ST, the biological night length described in the literature. Our results favour perennial ST and suggest assigning time-zones close to sun time to prevent social jetlag and sleep deprivation.

Biological responses of Chironomus sancticaroli to exposure to naturally aged PP microplastics under realistic concentrations.

Microplastic (MP) is yet another form of chronic anthropogenic contribution to the environment. MPs are plastic particles (<5 mm) that have been widely found in the most diverse natural environments, but their real impacts on ecosystems are still under investigation. Here, we studied the toxicity of naturally aged secondary polypropylene (PP) MPs after constant exposure to ultraviolet radiation (26 µm) to the third instar larvae of Chironomus sancticaroli, a dipteran species. The concentrations tested were 13.5; 67.5; and 135 items g-1 of dry sediment. C. sancticaroli organisms were investigated for fragment ingestion, mortality and changes in enzymatic biomarkers after 144 h of exposure. The organisms were able to ingest MPs from the first 48 h, and the amount of items internalized was dose-dependent and time-dependent. Overall, the results show that mortality was low, being significant at the lowest and highest concentrations (13.5 and 135 items g-1). Regarding changes in biochemical markers, after 144 h MDA and CAT activities were both significantly altered (increased and reduced, respectively), while SOD and GST levels were unchanged. In the present study, naturally aged polypropylene MPs induced biochemical toxicity in C. sancticaroli larvae, with toxicity being higher according to exposure time and particle concentration.

Epidemiological Impact of Neurodegenerative Diseases in the Rural Spanish-Portuguese Cross-Border Region.

INTRODUCTION: Epidemiological data indicate that neurodegenerative diseases show a high prevalence with a progressive increasing trend, especially in aging populations, as is the case in rural areas. The objective of this study was to assess the quantitative impact of neurodegenerative diseases in rural areas of the Spanish-Portuguese border region and to describe the epidemiological profile of the most prevalent disorders in one of the most depopulated and aged regions of Europe. METHODS: A cross-sectional descriptive study was designed to estimate the prevalence of subjects diagnosed with the most common neurodegenerative disorders: dementia (Alzheimer's disease and other dementias), Parkinson's disease and Parkinsonism, and multiple sclerosis in the Spanish-Portuguese cross-border border region in 2020. It includes Bragança and Guarda Districts (Portugal) and Salamanca (Castilla y León, Spain). RESULTS: Neurodegenerative diseases accounted for 1.85% in the Spanish-Portuguese cross-border region in 2020; a total of 5,819 records were reported: 987 (prevalence, 2.51%) in Salamanca (Spain); 2,332 (prevalence, 1.87%) in Bragança; and 2,500 (prevalence, 1.66%) in Guarda. Female population suffered from them in higher proportion (2.35 vs. 1.32%). Dementia represented 1.19% (3,744), Parkinson's disease and Parkinsonism 0.58% (1,823), and multiple sclerosis 0.08% (252). These disorders impacted older age groups. In the rural border region of Spain, 1 out of 4 cases were institutionalized. CONCLUSION: The findings reveal the health impact of neurodegenerative diseases in the Spanish-Portuguese cross-border region. The epidemiological data emphasize the region's circumstances and highlight research priorities. Intervention strategies must be implemented in the region to ensure quality healthcare in rural areas.

Sleep and violence perpetration: A review of biological and environmental substrates.

Violence is a worldwide societal burden that negatively impacts individual health, wellbeing and economic development. Evidence suggests a bidirectional relationship between sleep changes and violence. This review details, evaluates and discusses the biological and demographic substrates linking sleep and violence perpetration, and summarizes the overlap of brain areas, functional neuronal systems and genetic features involved, not including violent behaviours during sleep. Knowledge on the biological variables that affect the individual's susceptibility to violent behaviour may have implications for criminology, management of detentions and rehabilitation strategies.

Health-Related Behaviors and Perceived Addictions: Predictors of Depression During the COVID Lockdown.

ABSTRACT: The COVID-19 pandemic led to a worldwide implementation of measures to contain the virus from spreading, and social isolation was one of those measures. Social isolation is associated with increased susceptibility to various neuropsychiatric disorders, including depression. Because this risk varies between populations and their respective habits, understanding which behaviors play a positive or harmful role is fundamental for people with depression under these conditions. This study aims to 1) compare health-related behaviors (HRBs) and perceived addictions between healthy individuals and individuals who reported having a depressive disorder at the time of the first outbreak of the COVID-19 pandemic in Portugal, and 2) test which HRB and perceived addictions are associated with depression. Participants were a matched for age and sex and comprised 968 adults divided into two groups: healthy (484, 50%) and depressed (484, 50%). They completed online self-report questionnaires, and the data were analyzed in SPSS. Logistic regressions showed that being a health professional, getting up in a later time during weekends, and a higher consumption of soft drinks increased the risk for depression. In contrast, higher education and higher intensity of physical activity decreased this risk. Perceived addictions to TV, social networking, and gaming increased the risk for depression. HRBs and the identified addictions associated with depression during the confinement should be targeted in clinical and community interventions.

Health risk behaviors before and during COVID-19 and gender differences.

Changes in routines and habits, fear of contamination from the coronavirus disease 2019 (COVID-19) virus, and economic crisis have resulted in significant impacts upon individuals' lives, health, and risk behaviors. The present study aims to analyze health risk behaviors and gender differences of Portuguese adults before and during the COVID-19 pandemic. A quantitative analysis using SPSS v. 26 software presents the evaluation of 5746 responses (M = 48.5 years, SD = 14.3), of which 67.7% were female. t Test was used to study differences in means before and during the pandemic and analysis of variance test to analyze gender differences. In the comparative study before and during the pandemic showed a decrease in the number of meals per day, physical activity and perception of sleep quality; an increase in tobacco use, beer consumption, and media use (TV, mobile phone, social networks, and online games). Gender differences study demonstrated that the number of meals per day suffered a decrease from pre to pandemic in women, while increasing in men, becoming prominent in the second moment under study. Both genders had an increase in consumption behaviors and substance use, but women revealed a decrease in the consumption of wine during the pandemic, while men revealed more consumption behaviors in the variables under study. The use of media also changed, with men showing a higher level in TV hours per day, social networks and online games before the pandemic and in TV hours per day and games/online during the pandemic. Women stand out in the use of mobile phone per day during the pandemic. Daily physical activity decreased during the pandemic, as did sleep quality. Males revealed a higher practice of physical activity at both periods, as well as sleep quality. Based on the results presented, it is expected that considerations and actions in the scope of public health policies and health prevention and promotion, will be rethought and adapted to the specificities of each gender.

Ecological risk of imidacloprid on the Brazilian non-target freshwater organisms Chironomus sancticaroli and Poecilia reticulata.

Imidacloprid (IMI) is a neonicotinoid insecticide widely used in agriculture worldwide. This pesticide has been found in freshwater ecosystems, including Brazilian freshwaters. For this reason, studies are being conducted to detect the presence of IMI in freshwater and understand its effects on the aquatic biota. In the present study, the acute toxic effect of the imidacloprid commercial formulation (ICF) Galeão® on the Brazilian non-target aquatic organisms Chironomus sancticaroli and Poecilia reticulata was evaluated. Enzymatic activities (glutathione S-transferase (GST), catalase (CAT), and ascorbate peroxidase (APX)) were also determined. Moreover, we considered 11 studies that detected IMI concentrations up to 3.65 µg.L-1 in 28 different Brazilian freshwaters to evaluate the acute ecological risk of IMI in these environments. From the ecotoxicological assays, we determined the LC50 values for C. sancticaroli (LC50-48 h 1.52 µg.L-1) and P. reticulata (LC50-96 h 122.65 mg.L-1). The high sensitivity of C. sancticaroli demonstrates that this species could be used as a bioindicator in studies investigating the contamination of freshwater by IMI. Enzymatic activity changes were observed in both organisms and offered sublethal responses to the effects of the pollution by IMI on aquatic biota. Our results suggest that the presence of IMI in Brazilian aquatic ecosystems can represent a potential ecological risk for the aquatic insect populations and, consequently, cause an imbalance in these ecosystems. The present study provides relevant and comparable toxicity information that may be useful to develop public policies to protect the Brazilian aquatic ecosystem from IMI contamination.

Social jetlag, a novel predictor for high cardiovascular risk in blue-collar workers following permanent atypical work schedules.

Cardiovascular diseases cause >4 million deaths each year in Europe alone. Preventive approaches that do not only consider individual risk factors but their interaction, such as the Systematic COronary Risk Evaluation (SCORE), are recommended by European guidelines. Increased cardiovascular risk is associated with shift-work, surely interacting with the concurrent conditions: disruption of sleep, unhealthy behaviours, and circadian misalignment. Social jetlag (SJL) has been proposed as a way to quantify circadian misalignment. We therefore investigated the association between SJL and cardiovascular health in a cross-sectional observational study involving blue-collar workers, who either worked permanent morning, evening, or night shifts. Sociodemographic, health and productivity data were collected through questionnaires. Blood pressure and cholesterol were measured and the cardiovascular risk was estimated according to the relative risk SCORE chart. Bivariate analysis was performed according to the cardiovascular risk and the relationship between SJL and high cardiovascular risk was analysed through logistic regression. Cumulative models were performed, adjusted for various confounding factors. After 49 exclusions, the final sample comprised 301 workers (56% males; aged <40 years, 73%). Mean standard deviation (SD) SJL was 1:57 (1:38) hr (59.4% ≤2 hr). Cardiovascular risk was high in 20% of the sample. Multivariate analysis revealed SJL to be an independent risk factor for high cardiovascular risk. Each additional hour of SJL increased this risk by >30% (odds ratio 1.31, 95% confidence interval 1.02-1.68). This is the first study indicating that SJL potentially increases cardiovascular risk, and suggests that sleep and individual circadian qualities are critical in preventing negative health impacts of shift-work.

Validation of the Munich Actimetry Sleep Detection Algorithm for estimating sleep-wake patterns from activity recordings.

Periods of sleep and wakefulness can be estimated from wrist-locomotor activity recordings via algorithms that identify periods of relative activity and inactivity. Here, we evaluated the performance of our Munich Actimetry Sleep Detection Algorithm. The Munich Actimetry Sleep Detection Algorithm uses a moving 24-h threshold and correlation procedure estimating relatively consolidated periods of sleep and wake. The Munich Actimetry Sleep Detection Algorithm was validated against sleep logs and polysomnography. Sleep-log validation was performed on two field samples collected over 54 and 34 days (median) in 34 adolescents and 28 young adults. Polysomnographic validation was performed on a clinical sample of 23 individuals undergoing one night of polysomnography. Epoch-by-epoch analyses were conducted and comparisons of sleep measures carried out via Bland-Altman plots and correlations. Compared with sleep logs, the Munich Actimetry Sleep Detection Algorithm classified sleep with a median sensitivity of 80% (interquartile range [IQR] = 75%-86%) and specificity of 91% (87%-92%). Mean onset and offset times were highly correlated (r = .86-.91). Compared with polysomnography, the Munich Actimetry Sleep Detection Algorithm reached a median sensitivity of 92% (85%-100%) but low specificity of 33% (10%-98%), owing to the low frequency of wake episodes in the night-time polysomnographic recordings. The Munich Actimetry Sleep Detection Algorithm overestimated sleep onset (~21 min) and underestimated wake after sleep onset (~26 min), while not performing systematically differently from polysomnography in other sleep parameters. These results demonstrate the validity of the Munich Actimetry Sleep Detection Algorithm in faithfully estimating sleep-wake patterns in field studies. With its good performance across daytime and night-time, it enables analyses of sleep-wake patterns in long recordings performed to assess circadian and sleep regularity and is therefore an excellent objective alternative to sleep logs in field settings.

Sleep medicine catalogue of knowledge and skills - Revision.

The 'catalogue of knowledge and skills' for sleep medicine presents the blueprint for a curriculum, a textbook, and an examination on sleep medicine. The first catalogue of knowledge and skills was presented by the European Sleep Research Society in 2014. It was developed following a formal Delphi procedure. A revised version was needed in order to incorporate changes that have occurred in the meantime in the International Classification of Sleep Disorders, updates in the manual for scoring sleep and associated events, and, most important, new knowledge in sleep physiology and pathophysiology. In addition, another major change can be observed in sleep medicine: a paradigm shift in sleep medicine has taken place. Sleep medicine is no longer a small interdisciplinary field in medicine. Sleep medicine has increased in terms of recognition and importance in medical care. Consequently, major medical fields (e.g. pneumology, cardiology, neurology, psychiatry, otorhinolaryngology, paediatrics) recognise that sleep disorders become a necessity for education and for diagnostic assessment in their discipline. This paradigm change is considered in the catalogue of knowledge and skills revision by the addition of new chapters.

Responses of Aquatic Nontarget Organisms in Experiments Simulating a Scenario of Contamination by Imidacloprid in a Freshwater Environment.

Several studies have indicated the presence of the neonicotinoid insecticide imidacloprid (IMI) in aquatic ecosystems in concentrations up to 320.0 µg L-1. In the present study, we evaluated the effects of the highest IMI concentration detected in surface water (320.0 µg L-1) on the survival of Chironomus sancticaroli, Daphnia similis, and Danio rerio in three different scenarios of water contamination. The enzymatic activities of glutathione S-transferase (GST), catalase (CAT), and ascorbate peroxidase (APX) in D. rerio also were determined. For this evaluation, we have simulated a lotic environment using an indoor system of artificial channels developed for the present study. In this system, three scenarios of contamination by IMI (320.0 µg L-1) were reproduced: one using reconstituted water (RW) and the other two using water samples collected in unpolluted (UW) and polluted (DW) areas of a river. The results indicated that the tested concentration was not able to cause mortality in D. similis and D. rerio in any proposed treatment (RW, UW, and DW). However, C. sancticaroli showed 100% of mortality in the presence of IMI in the three proposed treatments, demonstrating its potential to impact the community of aquatic nontarget insects negatively. Low IMI concentrations did not offer risks to D. rerio survival. However, we observed alterations in GST, CAT, and APX activities in treatments that used IMI and water with no evidence of pollution (i.e., RW and UW). These last results demonstrated that fish are more susceptible to the effects of IMI in unpolluted environments.

The prevalence of excessive sleepiness is higher in shift workers than in patients with obstructive sleep apnea.

Excessive daytime sleepiness (EDS) is a common feature among shift workers as well as in obstructive sleep apnea (OSA) patients. There are several important accidents related to sleep disturbances causing EDS. The aim of this study was to evaluate EDS in a group of shift workers (regular rotating) from civil aviation and to compare them with OSA patients (n = 300) and with a group of regular workers (RW) (n = 140). Our sample was composed of 730 working-age individuals (aged 18-67 years). The regular rotating shift workers (SW) sample was composed of 290 aeronautical mechanics. EDS was evaluated with the Epworth Sleepiness Scale (ESS) and defined as a score ≥ 11. The prevalence value obtained for the EDS of RW was 37.1%, for SW it was 60.7% and for OSA patients it was 40.7%. A logistic regression model for EDS in a subsample composed of men and matched for age and BMI, controlling for self-reported sleep duration, showed an increased risk of EDS for SW (OR = 3.91, p = .001), with the RW group as reference. OSA patients did not differ from RW on EDS levels. This study emphasizes the presence of EDS in a shift work group of civil aviation professionals, which exceeded the EDS level of a positive control group of OSA patients. Sleep hygiene education for companies' workers and management is important and mitigation strategies should be implemented to reduce excessive sleepiness among workers.

Long-term performance and acute toxicity assessment of scaled-up air-cathode microbial fuel cell fed by dairy wastewater.

Long-term performance of a scaled-up air-cathode microbial fuel cell (MFC) and toxicity removal were studied with dairy wastewater (DW) used as the substrate. The MFC in a semi-continuous flow was strategically inoculated with consortium of Shewanella oneidensis and Clostridium butyricum. The scaled-up approach delivered a maximum power density of 0.48 W/m3 (internal resistance of 73 Ω) removing 93% of total chemical oxygen demand and 95% of total biochemical oxygen demand at organic loading rate (OLR) of 0.9 kg COD/m3/d and hydraulic retention time (HRT) of 21 days. It also achieved high removal efficiency of nitrate (100%), organic nitrogen (57%), sulfate (90%) and organic phosphorus (90%). The power generation and DW degradation performance decreased with OLR of 1.8 kg COD/m3/d and HRT of 10.5 days. Furthermore, testing of acute toxicity with the microcrustacean, Daphnia similis, revealed high toxic effect of the raw DW, but no toxic effects of the MFC effluent during 95 days of operation. These outcomes demonstrated that scaled-up MFC fed with high-strength DW should be an effective system for pollutants removal and simultaneously energy recovery.

Sedentary Time, Physical Activity, Fitness, and Physical Function in Older Adults: What Best Predicts Sleep Quality?

The aim of this study was to identify the best predictor of sleep quality (SQ) among physical behavior or capacity-related variables, namely physical activity, sedentary time, fitness, and physical function (activities of daily living) of independent elders using a representative sample of Portuguese aged 65 years and older (N = 437). SQ and activities of daily living were evaluated by a questionnaire, sedentary time, and physical activity through accelerometry, and physical fitness by means of the Senior Fitness Test. The logistic regression analysis revealed that activities of daily living measured by the Composite Physical Function was the only explanatory variable discriminating between poor SQ and good SQ. Receiver operating characteristic analysis showed that the best trade-off between sensitivity and specificity to discriminate older adults with poor SQ and good SQ was 20 points in the Composite Physical Function (sensitivity = 57.9%; specificity = 60.9%; area under the curve = 0.600, 95% confidence interval [0.536, 0.665], p = .003). Better physical function seems to be associated with better SQ in independent elders.

Sleep duration, body composition, dietary profile and eating behaviours among children and adolescents: a comparison between Portuguese acrobatic gymnasts.

Sleep, body composition and dietary intake are crucial for athletes' health and performance but have never been investigated in acrobatic gymnasts. Thus, this study aimed to evaluate sleep, body composition, dietary intake and eating behaviours of acrobatic gymnasts. Using a cross-sectional study, 82 Portuguese acrobatic gymnasts (12.8 ± 3.1 years, 61 females and 21 males) were evaluated during the competitive period to collect training data, sleep duration, body composition, dietary intake and eating behaviours before, during and after practices. Most of the gymnasts (91.5%) slept less than 8 h/night. Female adolescents slept significantly less on weekdays than female children (P = 0.024). Female adolescents' mean body mass was below the normal and 60.0% showed menstrual irregularities; 4.9% of females bases were overweight and 2.4% obese. Male adolescents slept significantly less on weekdays than male children (P = 0.001); significant differences were shown for fat-free mass (P = 0.014); however, 3.7% were overweight. All daily energy and macronutrient intakes were significantly different according to age and gender, with exception for fibre and energy intake in females (P = 0.057 and P = 0.052, respectively), and for protein in males (P = 0.068). Female and male adolescents demonstrated significant lower energy availability (32.8 ± 9.4 kcal/kg FFM/day and 45.1 ± 14.7 kcal/kg FFM/day) than children (45.8 ± 8.7 kcal/kg FFM/day and 53.8 ± 9.1 kcal/kg FFM/day), respectively. Significant low intakes of important vitamins and minerals were reported. Most of the participants did not eat or drink during or immediately after training sessions. CONCLUSION: Acrobatic gymnasts revealed low BF, short sleep duration and macro- and micronutrients inadequate intakes. The Female Athlete Triad was demonstrated by female adolescent gymnasts. What is Known: • Adequate dietary intake is an important resource for athletes' short and long-term health and performance. • There are no published studies in acrobatic gymnasts' sleep, body composition or dietary intake. What is New: • This study provides the first data on significant differences in energy intake and availability between acrobatic gymnasts; overweight and obesity were present in females. • Macro and micronutrients were inappropriate. Female Athlete Triad was observed in female adolescent acrobatic gymnasts.

Smajor Hemorrahage During Mediastinoscopy: Do You Panic or Do you Have a Protocol?

INTRODUCTION: Although uncommon, major vessel hemorrhage is the most feared complication of mediastinoscopy. Our goal was to determine the optimal management strategy and to develop a simple and accessible protocol for optimizing care in these situations. METHODS: Data collection after reviewing the relevant literature. A literature review was conducted using the following databases: PubMed, Medline, Embase and ScienceDirect. RESULTS: The protocol consists of three distinct parts - initial checklist, considerations in minor bleeding and performance in major bleeding. In this last section we propose an initial approach based mainly on fluid resuscitation and immediate surgical correction if the former has not been successful. CONCLUSION: Mediastinoscopy continues to be an important and effective diagnostic tool. However, it can cause important iatrogenic lesions which the anesthesiologist and surgical team must be prepared to diagnose early and treat properly.

Anaesthetic Management in a Duchennne Muscle Dystrophy Patient for Treatment of Recurrent Pneumothorax.

INTRODUCTION: Duchenne muscular dystrophy (DMD) is an x linked recessive disorder. Long term prognosis is ominous, with development of respiratory distress and cardiomyopathy in advanced stage of the disease and expected death in the teens-to-mid 20s due to respiratory or cardiac failure. Peri-operative management of this patients is challenging due to difficult airway anatomy (macroglossia, limited neck and mandibular mobility). Additionally, they are at risk of developing malignant hyperthermia, rhabdomyolysis and hyperkalemic cardiac arrest when exposed to halogenated inhalational anaesthetics and depolarizing muscle relaxants. METHODS: We describe a case of DMD proposed to a thoracotomy for treatment of recurrent pneumothorax and its anaesthetic approach. RESULTS: A 22-year-old male patient with DMD presented at emergency department due dyspnoea starting at 3 days associated with right scapular pain, enhanced by breathing. The patient already presents with mild cardiomyopathy (ejection fraction of 55%, mild mitral and tricuspid regurgitation), severe restrictive respiratory defect, requiring continuous BiPAP. The patient was markedly denourished (BMI of 12 kg/m2) and presented with nearly absent breathing sounds on the right side. Chest radiography showed large pneumothorax on the right side with no signs of tension. Drainage was performed. Despite initial success, recurrence of pneumothorax occurred on the several attempts of clamping. A bronchopleural fistula was suspected and operative treatment was considered. Considering the comorbidities, he was graded ASA IV with a difficult airway due to macroglossia, limited neck and mandibular mobility. Oro-tracheal intubation was performed with slight sedation (propofol, without neuromuscular blocks). Difficult airway anatomy (direct laryngoscopy - Cormack 4) successfully approached with a bougie and Mccoy blade. Fibreoptic intubation approach was immediately available in the operating room, if required. Total intravenous anaesthesia was decided (remifentanil and propofol, administered by continuous infusion, without neuromuscular blockers). Volume controlled protective ventilation as used (tidal volume 6-8ml/kg, respiratory frequency of 14-16/ min; FiO2: 0,5). No bronchopleural fistula was detected and pleurodesis was performed with biologic glue. Patient remained intubated and was transferred to the ICU for monitoring, having been discharged on the 2nd day to the ward. Despite this, pneumothorax recurrence occurred, and surgery was performed again, using the same anaesthetic approach, this time with successful closure of the bronchopleural fistula. CONCLUSION: Total intravenous anaesthesia, without neuromuscular blockers, is a safe and effective option for DMD patients. Anaesthesiologists must consider the possibility of cardio-pulmonary disabilities, difficult airway management, as well as the high risk of malignant hyperthermia in these patients.

The European Narcolepsy Network (EU-NN) database.

Narcolepsy with cataplexy is a rare disease with an estimated prevalence of 0.02% in European populations. Narcolepsy shares many features of rare disorders, in particular the lack of awareness of the disease with serious consequences for healthcare supply. Similar to other rare diseases, only a few European countries have registered narcolepsy cases in databases of the International Classification of Diseases or in registries of the European health authorities. A promising approach to identify disease-specific adverse health effects and needs in healthcare delivery in the field of rare diseases is to establish a distributed expert network. A first and important step is to create a database that allows collection, storage and dissemination of data on narcolepsy in a comprehensive and systematic way. Here, the first prospective web-based European narcolepsy database hosted by the European Narcolepsy Network is introduced. The database structure, standardization of data acquisition and quality control procedures are described, and an overview provided of the first 1079 patients from 18 European specialized centres. Due to its standardization this continuously increasing data pool is most promising to provide a better insight into many unsolved aspects of narcolepsy and related disorders, including clear phenotype characterization of subtypes of narcolepsy, more precise epidemiological data and knowledge on the natural history of narcolepsy, expectations about treatment effects, identification of post-marketing medication side-effects, and will contribute to improve clinical trial designs and provide facilities to further develop phase III trials.

24-hour rhythmicity of seizures in refractory focal epilepsy.

The occurrence of seizures in specific types of epilepsies can follow a 24-hour nonuniform or nonrandom pattern. We described the 24-hour pattern of clinical seizures in patients with focal refractory epilepsy who underwent video-electroencephalography monitoring. Only patients who were candidates for epilepsy surgery with an unequivocal seizure focus were included in the study. A total of 544 seizures from 123 consecutive patients were analyzed. Specific time of seizures were distributed along 3- or 4-hour time blocks or bins throughout the 24-hour period. The mean age of the subjects was 37.7 years, with standard deviation of 11.5 years, median of 37. The majority were females (70/56%). The majority of patients had a seizure focus located in the mesial temporal lobe (102/83%) and in the neocortical temporal lobe (13/11%). The remaining patients had a seizure focus located in the extratemporal lobe (8/6%). The most common etiology was mesial temporal sclerosis (86/69.9%). Nonuniform seizure distribution was observed in seizures arising from the temporal lobe (mesial temporal lobe and neocortical temporal lobe), with two peaks found in both 3- and 4-hour bins: 10:00-13:00/16:00-19:00 and 08:00-12:00/16:00-20:00 respectively (p=0.004). No specific 24-hour pattern was identified in seizures from extratemporal location. The 24-hour rhythmicity of seizure distribution is recognized in certain types of epilepsy, but studies on the topic are scarce. Their replication and validation is therefore needed. Our study confirms the bimodal pattern of temporal lobe epilepsy independently of the nature of the lesion. However, peak times differ between different studies, suggesting that the ambient, rhythmic exogenous factors or environmental/social zeitgebers, may modulate the 24-hour rhythmicity of seizures. Characterization of these 24-hour patterns of seizure occurrence can influence diagnosis and treatment in selected types of epilepsy, such as the case of temporal lobe epilepsy, the most common drug-resistant epilepsy.