Epidemiology and outcome of idiopathic generalized epilepsy in adults.

BACKGROUND AND PURPOSE: Drug-resistant idiopathic generalized epilepsy (IGE) remains challenging despite a favourable overall prognosis of IGE, and little is known about basic epidemiology and long-term outcome of drug-resistant IGE. The aim of the study was to describe the incidence, prevalence and outcome of IGE in an unbiased, population-based cohort. METHODS: In 2014-2018, all patients (≥17 years) with IGE inhabiting the island of Funen (496 000 inhabitants) were recruited. The socioeconomic and clinical information available for 406 individuals was assessed. Median follow-up was 15 years. RESULTS: The average IGE incidence (2008-2017) was 2.9/100 000 inhabitants/year. The point prevalence of identifiable IGE patients was 1.0/1000 adults (juvenile myoclonic epilepsy 0.4/1000; absence epilepsy 0.3/1000, epilepsy with generalized tonic-clonic seizures alone 0.3/1000); 92.1% of the patients were diagnosed before 25 years of age. When correcting for unequal age distribution in the cohort, 1102 people on the island of Funen fulfilled the diagnostic criteria for IGE at the age of 25 (estimated prevalence 2.7/1000 adults). In the year before data closure, 121 patients reported seizures. Fifty patients met the definition of drug-resistant IGE (12.1% of the cohort, 4.5% of the estimated 1102 IGE patients). The average seizure burden of all patients with drug-resistant IGE was 2.2 generalized tonic-clonic seizures per year; only 14 patients suffered more than two generalized tonic-clonic seizures per year. Drug-resistant IGE was associated with an increased risk of requiring treatment for affective disorders and a reduced probability of working full time. CONCLUSION: Idiopathic generalized epilepsy was associated with a low risk of persistent drug-resistant seizures requiring specialist medical attention. Drug resistance was associated with a negative socioeconomic outcome.

Effects of acidic deposition and soil acidification on sugar maple trees in the Adirondack Mountains, New York.

We documented the effects of acidic atmospheric deposition and soil acidification on the canopy health, basal area increment, and regeneration of sugar maple (SM) trees across the Adirondack region of New York State, in the northeastern United States, where SM are plentiful but not well studied and where widespread depletion of soil calcium (Ca) has been documented. Sugar maple is a dominant canopy species in the Adirondack Mountain ecoregion, and it has a high demand for Ca. Trees in this region growing on soils with poor acid-base chemistry (low exchangeable Ca and % base saturation [BS]) that receive relatively high levels of atmospheric sulfur and nitrogen deposition exhibited a near absence of SM seedling regeneration and lower crown vigor compared with study plots with relatively high exchangeable Ca and BS and lower levels of acidic deposition. Basal area increment averaged over the 20th century was correlated (p < 0.1) with acid-base chemistry of the Oa, A, and upper B soil horizons. A lack of Adirondack SM regeneration, reduced canopy condition, and possibly decreased basal area growth over recent decades are associated with low concentrations of nutrient base cations in this region that has undergone soil Ca depletion from acidic deposition.

Effects of elevated CO2, warming and drought episodes on plant carbon uptake in a temperate heath ecosystem are controlled by soil water status.

The impact of elevated CO2, periodic drought and warming on photosynthesis and leaf characteristics of the evergreen dwarf shrub Calluna vulgaris in a temperate heath ecosystem was investigated. Photosynthesis was reduced by drought in midsummer and increased by elevated CO2 throughout the growing season, whereas warming only stimulated photosynthesis early in the year. At the beginning and end of the growing season, a T × CO2 interaction synergistically stimulated plant carbon uptake in the combination of warming and elevated CO2. At peak drought, the D × CO2 interaction antagonistically down-regulated photosynthesis, suggesting a limited ability of elevated CO2 to counteract the negative effect of drought. The response of photosynthesis in the full factorial combination (TDCO2) could be explained by the main effect of experimental treatments (T, D, CO2) and the two-factor interactions (D × CO2, T × CO2). The interactive responses in the experimental treatments including elevated CO2 seemed to be linked to the realized range of treatment variability, for example with negative effects following experimental drought or positive effects following the relatively higher impact of night-time warming during cold periods early and late in the year. Longer-term experiments are needed to evaluate whether photosynthetic down-regulation will dampen the stimulation of photosynthesis under prolonged exposure to elevated CO2.

Interactive effects of elevated CO2, warming, and drought on photosynthesis of Deschampsia flexuosa in a temperate heath ecosystem.

Global change factors affect plant carbon uptake in concert. In order to investigate the response directions and potential interactive effects, and to understand the underlying mechanisms, multifactor experiments are needed. The focus of this study was on the photosynthetic response to elevated CO(2) [CO2; free air CO(2) enrichment (FACE)], drought (D; water-excluding curtains), and night-time warming (T; infrared-reflective curtains) in a temperate heath. A/C(i) curves were measured, allowing analysis of light-saturated net photosynthesis (P(n)), light- and CO(2)-saturated net photosynthesis (P(max)), stomatal conductance (g(s)), the maximal rate of Rubisco carboxylation (V(cmax)), and the maximal rate of ribulose bisphosphate (RuBP) regeneration (J(max)) along with leaf δ(13)C, and carbon and nitrogen concentration on a monthly basis in the grass Deschampsia flexuosa. Seasonal drought reduced P(n) via g(s), but severe (experimental) drought decreased P(n) via a reduction in photosynthetic capacity (P(max), J(max), and V(cmax)). The effects were completely reversed by rewetting and stimulated P(n) via photosynthetic capacity stimulation. Warming increased early and late season P(n) via higher P(max) and J(max). Elevated CO(2) did not decrease g(s), but stimulated P(n) via increased C(i). The T×CO2 synergistically increased plant carbon uptake via photosynthetic capacity up-regulation in early season and by better access to water after rewetting. The effects of the combination of drought and elevated CO(2) depended on soil water availability, with additive effects when the soil water content was low and D×CO2 synergistic stimulation of P(n) after rewetting. The photosynthetic responses appeared to be highly influenced by growth pattern. The grass has opportunistic water consumption, and a biphasic growth pattern allowing for leaf dieback at low soil water availability followed by rapid re-growth of active leaves when rewetted and possibly a large resource allocation capability mediated by the rhizome. This growth characteristic allowed for the photosynthetic capacity up-regulations that mediated the T×CO2 and D×CO2 synergistic effects on photosynthesis. These are clearly advantageous characteristics when exposed to climate changes. In conclusion, after 1 year of experimentation, the limitations by low soil water availability and stimulation in early and late season by warming clearly structure and interact with the photosynthetic response to elevated CO(2) in this grassland species.

Rifting of the oceanic Azores Plateau with episodic volcanic activity.

Extension of the Azores Plateau along the Terceira Rift exposes a lava sequence on the steep northern flank of the Hirondelle Basin. Unlike typical tholeiitic basalts of oceanic plateaus, the 1.2 km vertical submarine stratigraphic profile reveals two successive compositionally distinct basanitic to alkali basaltic eruptive units. The lower unit is volumetrically more extensive with ~ 1060 m of the crustal profile forming between ~ 2.02 and ~ 1.66 Ma, followed by a second unit erupting the uppermost ~ 30 m of lavas in ~ 100 kyrs. The age of ~ 1.56 Ma of the youngest in-situ sample at the top of the profile implies that the 35 km-wide Hirondelle Basin opened after this time along normal faults. This rifting phase was followed by alkaline volcanism at D. João de Castro seamount in the basin center indicating episodic volcanic activity along the Terceira Rift. The mantle source compositions of the two lava units change towards less radiogenic Nd, Hf, and Pb isotope ratios. A change to less SiO2-undersaturated magmas may indicate increasing degrees of partial melting beneath D. João de Castro seamount, possibly caused by lithospheric thinning within the past 1.5 million years. Our results suggest that rifting of oceanic lithosphere alternates between magmatically and tectonically dominated phases.

Carbon and nitrogen cycles in European ecosystems respond differently to global warming.

The global climate is predicted to become significantly warmer over the next century. This will affect ecosystem processes and the functioning of semi natural and natural ecosystems in many parts of the world. However, as various ecosystem processes may be affected to a different extent, balances between different ecosystem processes as well as between different ecosystems may shift and lead to major unpredicted changes. In this study four European shrubland ecosystems along a north-south temperature gradient were experimentally warmed by a novel nighttime warming technique. Biogeochemical cycling of both carbon and nitrogen was affected at the colder sites with increased carbon uptake for plant growth as well as increased carbon loss through soil respiration. Carbon uptake by plant growth was more sensitive to warming than expected from the temperature response across the sites while carbon loss through soil respiration reacted to warming in agreement with the overall Q10 and response functions to temperature across the sites. Opposite to carbon, the nitrogen mineralization was relatively insensitive to the temperature increase and was mainly affected by changes in soil moisture. The results suggest that C and N cycles respond asymmetrically to warming, which may lead to progressive nitrogen limitation and thereby acclimation in plant production. This further suggests that in many temperate zones nitrogen deposition has to be accounted for, not only with respect to the impact on water quality through increased nitrogen leaching where N deposition is high, but also in predictions of carbon sequestration in terrestrial ecosystems under future climatic conditions. Finally the results indicate that on the short term the above-ground processes are more sensitive to temperature changes than the below ground processes.

Root-surface phosphatase activity in shrublands across a European gradient: effects of warming.

Root-surface phosphatase activities were measured in natural and semi-natural shrublands across an European climatic gradient of temperature and rainfall including Wales (WL), Denmark (DK), Netherlands (NL), Hungary (HU), Italy (IT) and Spain (SP). In each site a warming experiment was conducted since 1999 or 2001 by means of passive night-time warming using reflective curtains that covered the vegetation at night. The treatments increased yearly average soil temperatures around 0. 8 degrees C in most of sites. Root-surface phosphatase activity values ranged between 56 mg PNP g(-1) h(-1) in IT and 3.5 mg PNP g(-1) h(-1) in HU. Warming had no effect on root-surface phosphatase activity across the sites and only in Hungary a slight increase was detected. Plants at Mediterranean sites (IT, SP) showed a higher root-surface phosphatase activity than plants at temperate sites (WL, NL, DK). We suggest it might be an adaptation of plant species evolved under Mediterranean climate that allows them a) to compensate in wet period for the decrease in phosphatase activity, and thus P uptake, during drought periods, and/or b) to benefit from soluble organic P flushes following the frequent drying-rewetting episodes experienced by soils in Mediterranean ecosystems.

Challenges in quantifying biosphere-atmosphere exchange of nitrogen species.

Recent research in nitrogen exchange with the atmosphere has separated research communities according to N form. The integrated perspective needed to quantify the net effect of N on greenhouse-gas balance is being addressed by the NitroEurope Integrated Project (NEU). Recent advances have depended on improved methodologies, while ongoing challenges include gas-aerosol interactions, organic nitrogen and N(2) fluxes. The NEU strategy applies a 3-tier Flux Network together with a Manipulation Network of global-change experiments, linked by common protocols to facilitate model application. Substantial progress has been made in modelling N fluxes, especially for N(2)O, NO and bi-directional NH(3) exchange. Landscape analysis represents an emerging challenge to address the spatial interactions between farms, fields, ecosystems, catchments and air dispersion/deposition. European up-scaling of N fluxes is highly uncertain and a key priority is for better data on agricultural practices. Finally, attention is needed to develop N flux verification procedures to assess compliance with international protocols.

Effects of systemic NO synthase inhibition on choroidal and optic nerve head blood flow in healthy subjects.

PURPOSE: There is evidence from animal studies that nitric oxide (NO) is a major determinant of ocular blood flow. In humans NO synthase inhibition reduces pulsatile choroidal blood flow, but no data on optic nerve head (ONH) vasculature are available yet. The goal of this study was to investigate the effects of NO synthase inhibition on human choroidal and ONH blood flow using laser Doppler flowmetry. METHODS: The study design was a randomized, placebo-controlled, double-masked, balanced three-way crossover. On separate study days 12 healthy male subjects received infusions of N:(G)-nitro-L-arginine (L-NMMA; either 3 mg/kg over 5 minutes followed by 30 microg/kg per minute over 55 minutes or 6 mg/kg over 5 minutes followed by 60 microg/kg per minute over 55 minutes) or placebo. The effects of L-NMMA or placebo on choroidal and ONH blood flow were measured with laser Doppler flowmetry. In addition, laser interferometric measurement of fundus pulsation was performed in the macula to assess pulsatile choroidal blood flow. RESULTS: L-NMMA reduced all outcome parameters in the choroid and the ONH. The higher dose of L-NMMA caused a significant decrease in blood flow in the choroid (-26% +/- 9%; P: < 0.001) and the ONH (-20% +/- 16%; P: < 0.001) as evidenced from laser Doppler flowmetry and a significant decrease in fundus pulsation amplitude (-26% +/- 5%; P: < 0.001). CONCLUSIONS: These results indicate that NO is continuously released in human choroidal and ONH vessels.

Efficacy of erbium:YAG laser ablation in Darier disease and Hailey-Hailey disease.

BACKGROUND: Among different surgical approaches, dermabrasion and carbon dioxide laser vaporization have been used to treat Hailey-Hailey disease (HHD) (familial benign chronic pemphigus) and Darier disease (DD) (keratosis follicularis), with various results. Because of the erbium: YAG laser's unique absorption characteristics in tissue water, erbium:YAG laser ablation combines the advantages of both techniques, avoiding thermal injury of vaporization and also allowing selectively deeper tissue removal in the follicular lesions of DD. Therefore, good results should be expected in both types of acantholytic disorders. OBSERVATIONS: Four patients (2 with HHD and 2 with DD) with different affected areas were treated with laser ablation. During a follow-up period ranging from 8 to 20 months, complete remission was achieved in 3 patients--2 with DD and 1 with HHD--and significant improvement was achieved in 1 patient with HHD. Histological examination of control biopsy specimens after ablation in 1 patient with DD revealed no signs of the disease and only a slight fibrosis in the papillary dermis. CONCLUSIONS: Erbium:YAG laser ablation effectively removes lesions of both HHD and DD and can also yield excellent long-term results in chronic, recalcitrant cases.

Atmospheric deposition to the edge of a spruce forest in Denmark.

Atmospheric deposition was measured during 1 year at the forest edge of a Norway spruce stand in Denmark. Inside the forest the deposition of H(+), Ca(2+), Mg(2+), Na(+), K(+), Cl(-), NO(3)(-), NH(4)(2) and SO(4)(2-) with canopy throughfall varies with the distance from the forest edge. The deposition at the edge is found to be 10-20 times as high as deposition to an open field and 2-8 times as high as deposition inside the stand. An exponential decrease in deposition as a function of the distance from the forest edge is found. Increased deposition of K(+) and non-sea salt Mg(2+), which mainly originates as a result of leaching from the needles may be explained by a larger leaf area index (LAI) at the forest edge. Deposition of particulate substances, especially Na(+), Cl(-), Mg(2+) and to some extent SO(4)(2-), NH(4)(+) and NO(3)(-) is increased much more than the LAI, which we believe to be caused by changes in wind movements at the forest edge.

Spatial variability of throughfall fluxes in a spruce forest.

The spatial variability of throughfall deposition of H(+), Ca(2+), Mg(2+), Na(+), K(+), Cl(-), NO(3)(-), NH(4)(+), O(4)(2-) to a Norway spruce (Picea abies (L.) Karst.) forest was intensively examined during the period October 1986 to October 1987. Large systematic spatial variability of the atmospheric deposition within the forest was observed. The flux of throughfall water was higher away from the trunk compared to the flux close to the trunk. In contrast to this, the deposition of all substances was considerably higher close to the trunk compared to the deposition at the periphery of the canopy. A linear decrease in deposition as a function of the distance from the nearest tree trunk was found. Further, the deposition varied quite dramatically between trees according to their size. The observed spatial variability in throughfall may be due to variabilities in the processes taking part in altering the distribution and composition of the precipitated water as it moves through the canopy. The influence of these processes of precipitation, wash-off, dry deposition and canopy exchange is discussed, and it is found that both increased dry deposition and canopy exchange in the tree tops contribute to the higher solute fluxes found close to the tree trunk.

Resilience and vulnerability of northern regions to social and environmental change.

The arctic tundra and boreal forest were once considered the last frontiers on earth because of their vast expanses remote from agricultural land-use change and industrial development. These regions are now, however, experiencing environmental and social changes that are as rapid as those occurring anywhere on earth. This paper summarizes the role of northern regions in the global system and provides a blueprint for assessing the factors that govern their sensitivity to social and environmental change.

Nitrogen cycling in a Norway spruce plantation in Denmark--a SOILN model application including organic N uptake.

A dynamic carbon (C) and nitrogen (N) circulation model, SOILN, was applied and tested on 7 years of control data and 3 years of manipulation data from an experiment involving monthly N addition in a Norway spruce ( Picea abies, L. Karst) forest in Denmark. The model includes two pathways for N uptake: (1) as mineral N after mineralisation of organic N, or (2) directly from soil organic matter as amino acids proposed to mimic N uptake by mycorrhiza. The model was parameterised and applied to the data from the control plot both with and without the organic N uptake included. After calibration, the models performance was tested against data from the N-addition experiment by comparing model output with measurements. The model reproduced well the overall trends in C and N pools and the N concentrations in soil solutions in the top soil layers whereas discrepancies in soil-solution concentrations in the deeper soil layers are seen. In the control data, the needle-N concentration was well reproduced except for small underestimations in some years because of drought effects not included in the model. In the N-addition experiment, SOILN reproduces the observed changes; in particular, the changes in needle-N concentrations and the overall distribution within the ecosystem of the extra added 3.5 g N m(-2) year(-1) parallel the observations. When organic N uptake is included, the simulations indicate that in the control plot receiving c. 1.9 g N m(-2) year(-1), the organic N uptake in average supplies 35% of the total plant N uptake. By addition of an extra 35 kg N ha(-1) year(-1), the organic N uptake is reduced to 16% of the total N uptake. Generally, inclusion of the pathway for organic N uptake improves model performance compared with observations for both C and N. This is because mineral N uptake alone implies a larger mineralisation rate, leading to bigger concentrations of N in the soil and soil water, bigger N losses, and net loss of c. 100 kg C ha(-1) year(-1), thereby causing depletion of the organic soil layer.

Root growth and N dynamics in response to multi-year experimental warming, summer drought and elevated CO2 in a mixed heathland-grass ecosystem.

Ecosystems exposed to elevated CO2 are often found to sequester more atmospheric carbon due to increased plant growth. We exposed a Danish heath ecosystem to elevated CO2, elevated temperature and extended summer drought alone and in all combinations in order to study whether the expected increased growth would be matched by an increase in root nutrient uptake of NH4+-N and NO3- -N. Root growth was significantly increased by elevated CO2. The roots, however, did not fully compensate for the higher growth with a similar increase in nitrogen uptake per unit of root mass. Hence the nitrogen concentration in roots was decreased in elevated CO2, whereas the biomass N pool was unchanged or even increased. The higher net root production in elevated CO2 might be a strategy for the plants to cope with increased nutrient demand leading to a long-term increase in N uptake on a whole-plant basis. Drought reduced grass root biomass and N uptake, especially when combined with warming, but CO2 was the most pronounced main factor effect. Several significant interactions of the treatments were found, which indicates that the responses were nonadditive and that changes to multiple environmental changes cannot be predicted from single-factor responses alone.

[Dermatitis artefacta presenting as photodermatosis]. / Dermatitis artefacta unter dem Bild einer Lichtdermatose.

Dermatitis artefacta is a form of a self-injury due to psychiatric disorders or internal conflicts. Delayed diagnosis often leads to unnecessary treatments. A 17-year old girl was referred with a putative photodermatosis presenting with erosions on an erythematous base on the face and forearms. The unusual rapid onset of new lesions following phototesting and in particular a reaction induced after a simulated light exposure substantiated the diagnosis of dermatitis artefacta. Faced with the diagnosed the patient admitted she had induced the lesions and was referred for psychiatric care.

[Postoperative pyoderma gangrenosum of Cullen]. / Postoperatives Pyoderma gangraenosum Cullen.

The clinical picture in pyoderma gangrenosum varies but a typical medical history with resistance to antimicrobial treatment and worsening or first manifestation of disease because of surgical procedures are indications of this diagnosis. We describe the course of a woman patient who had a pyoderma gangrenosum for more than 1.5 years. After confirming the diagnosis an immunomodulating therapy was initiated until complete remission of the ulcers. Differential diagnosis and different clinicopathologic forms of pyoderma gangrenosum are discussed and an overview of the association with internal diseases is provided.

[Attempted suicide with L-thyroxine in an adolescent girl]. / Intoxikation mit L-Thyroxin in suizidaler Absicht bei einer Jugendlichen.

BACKGROUND: Attempted suicide with l-thyroxine (LT4) is very rare, only being published in some case reports concerning young women. Experiences in the management of LT4 intoxication have already been made because overdosage in infants occurs more often. Guidelines and recommendations depend on the quantity of LT4 that has been ingested. PATIENT: A 17-year-old girl presented to the pediatric emergency department after intentional ingestion of 20 mg of LT4 20 hours prior to admission. Anamnestically she had increased the thyroxine dose rate up to 800 microg per day during the previous 4 months in order to obtain loss of weight. In spite of massive overdosage and extremely high serum thyroxine levels (fT4 794 pg/ml, TSH suppressed) the girls showed low symptoms and a mild clinical course. Because of tachycardia, hyperhidrosis and muscle tremor propranolol was applied. During the following 22 days fT4 levels returned to normal range and the adolescent was remitted to the outpatient clinic of the department of psychiatry. Whereas patients with graves disease and thyreotoxicosis present with severe symptoms our patient showed a mild clinical course in spite of having extremely high fT4 levels. CONCLUSIONS: The reasons are ambiguous. A possible answer could be an adaptation in higher fT4 levels in cause of the reported chronic LT4 overdosage.

Sodium thiosulphate as a promising therapeutic option to treat calciphylaxis.

A 35-year-old haemodialysis-dependent woman with chronic renal failure developed large, very painful necrotic ulcers and necrosis on the thighs, buttocks and the abdomen with signs of fast progression. The skin biopsy specimens showed a broad necrosis of the epidermis and thrombosed dermal vessels with focal calcium deposits within the wall. In addition, laboratory findings presented an increased product of serum calcium and phosphate concentrations. Thus, we diagnosed calciphylaxis on the basis of clinical, biochemical and histopathological criteria. We initiated a therapy in which our patient was treated with intravenous sodium thiosulphate 3 times weekly. Already after 2 weeks of treatment, no new lesions were detectable and there was a dramatic pain relief. In the following 4 weeks, a successive decline of the ulcers and the healing of individual tissue defects could be seen. Four months after the start of the therapy, the patient underwent successful renal transplantation. Thus, the intravenous therapy of calciphylaxis with sodium thiosulphate might be a new effective alternative in the treatment of this life-threatening disease.

Effects of who-ecosystem manipulations on ecosystem internal processes.

Whole-ecosystem manipulation is a useful tool for investigating the effects of air pollution, air pollution reduction strategies and management practices on the health and productivity of forests, and has become widely used in forest ecosystem research. This review of the whole-ecosystem manipulation research gives an overview of the recent and ongoing research within this field and synthesizes the results obtained so far.