Does age of onset in essential tremor have a bimodal distribution? data from a tertiary referral setting and a population-based study

Background/Aims: The distribution of age of onset of essential tremor (ET) is unclear, with discrepancies in the literature. Some data suggest a bimodal distribution and other data 1 late-life peak. We studied age of ET onset in 2 distinct settings: a population-based study and a tertiary referral center. Methods: Age of onset data were collected. Results: In the population, there was only a small peak at the age of ≤30 years (14.1% of cases) but a clear peak in later life (85.9% of cases). In the tertiary referral center, a bimodal distribution was apparent with 1 large peak (42.2% of cases) at the age of ≤40 years and the second large peak (57.8% of cases) in later life. Familial cases accounted for only 52.6% of young-onset cases from the population, yet 82.7% from the tertiary center. Discussion: In the population-based study, a peak in later life was clearly present but a young-onset peak was barely discernable, comprising few cases. By contrast, in a tertiary referral center, age of onset was clearly bimodal. While age of ET onset is often said to be bimodal, this may be due to the preferential referral to tertiary centers of patients with young-onset, familial ET

Isolated head tremor : part of the clinical spectrum of essential tremor? data from population-based and clinic-based case samples

Essential tremor (ET) still remains a clinical diagnosis. Nonetheless, it is misdiagnosed in 30 to 50% of cases. There are a number of areas of diagnostic uncertainty. One of these is isolated head tremor, on which published data are limited and at variance. We studied the prevalence of isolated head (i.e., neck) tremor in ET in two population-based studies (Turkey and New York) and a large clinical sample (New York); these 583 ET cases all received the same detailed tremor examination. Head tremor with mild arm tremor occurred in a very small percentage of cases in each sample (1.9 - 3.1%, overall 2.7%). Nearly all of them were women. Head tremor in the complete absence of arm tremor was not observed in any cases (0.0%). These clinical data may be of value to clinicians in practice settings and researchers in phenotyping efforts in the emerging field of ET genetics

Designing research tools : empirical knowledge as a base for future beekeeping.

The world-wide phenomenon of the disappearance of bees with the consequential imbalance of ecosystems is a problem that needs to be addressed in order to guarantee a sustainable future. Beekeeping is an activity, generally associated to agriculture, that allows small farmers to generate more food and income, this reality has had a shift in recent years with the number of small beekeepers decreasing, and big corporations gaining control over the industry. Regulations and the Colony Collapse Disorder, among other factors, may have a major role in these phenomena. With the intent to contribute to a more sustainable beekeeping practice, this research is being conducted to better understand this reality, looking for design driven opportunities. The challenge is to gain a deeper understanding of the beekeepers, a practice that is understudied by designers, particularly considering in-depth research methods. This paper discusses an experimental design study in the Turkish Aegean region that was conducted with local beekeepers to better understand the existing specifications: by discussing their business models, in order to understand and analyze socio-cultural factors that contributed to the existing system, and propose changes for a more sustainable practice. As a starting point for a first phase survey, the intent is to generate original data which will serve as the foundation for future works. Beekeepers in this scenario are mainly senior male farmers that belong to a low literacy audience, who learned the practice from their predecessors. In order to identify the problems of beekeepers, we needed to design and adjust our research methods according to the specifications of the audience. For this reason, a three stage inquiry was designed to better communication in order to extract measurable data from their empirical knowledge through a series of workshops. The case study reports the workshop sessions for identifying the design strategies with a focus on voicing the beekeepers problems. The first level draws upon explicit knowledge to identify the audience. For the second level an illustrated questionnaire was designed to visually represent the quantitative data about the business. The process is exercised with a new design because the content of knowledge is tacit and personal. Finally, the third level is an interview session to validate the data gathered from the previous stages. This paper will present the findings of the workshops with the aim to formulate an appropriate way to employ new approaches to exploring and defining design problems with visual representations. We also seek to extend our research in other contexts that will help discuss the role of design as a tool for developing new methodologies of design research. As a consequence we will propose prototypes of artifacts that will consider the implementation into beekeeping of new biomaterials, technology, branding and commercialization planning, etc., that may help create a more sustainable beekeeping practice. Beekeepers will be invited to analyze proposals and give their contributions. This process will render into redesigned optimized artifacts that will merge empirical knowledge with the needs of sustainable beekeeping. Keywords design research tools, beekeeping, sustainability, eco-innovation, cooperatio

Static Analysis of a Masonry Arched and Buttressed Retaining Wall

The wall of Casa Salvans is a masonry arched and buttressed retaining wall which was built in 1909 and located in Terrassa, Catalonia. It contains a series of 11 arches of unique shapes, dimensions and inclinations that are supported on buttresses. Despite being a common retaining wall typology, it can be very challenging to assess the stability of such structures given the variability of their geometry. As such, a systematic methodology for the stability assessment of such walls was developed and applied to the case of the wall of Casa Salvans. Self-produced photogrammetry survey enabled creating accurate three-dimensional models of the front of the wall that were later combined with available information from a topographic survey to build cross sections of the wall for analysis. Modern retaining wall design principles evaluating the stability against overturning and sliding were used to assess the condition of the wall. As a consequence of the uncertainties related to the soil parameters and buried elements of the wall, the stability factors could not comply with modern design criterion. Some parametric analyses were carried out on the shear strength parameters of the backfill, revealing that slight increases in the soil internal friction and wall friction angles enhance the stability factors significantly. Since reasonable soil internal and wall friction angles justifying the stability of the wall were obtained, carrying out a geotechnical survey to determine the actual values was recommended. The in-plane stability of the arches was also verified according to the Lower Bound Theorem by locating a thrust line that fits within all the boundaries despite very conservative geometric assumptions

Elevated Blood Lead Concentrations in Essential Tremor: A Case–Control Study in Mersin, Turkey

Essential tremor (ET) is one of the most common neurologic disorders. Aside from underlying susceptibility genes, recent studies have also begun to focus on environmental toxic factors. Yet there remains a paucity of information on such factors, making studies of environmental factors important. A recent study in New York City found blood lead concentrations to be elevated in ET cases compared with matched controls. Chronic exposure to lead produces cerebellar damage, and this could predispose individuals to develop ET

Diurnal and nocturnal drooling in Parkinson’s disease

Drooling as symptom of Parkinson’s disease (PD) has thus far been poorly defined. This uncertainty is reflected by high variations in published prevalence rates. The aim of this study was to investigate the prevalence of saliva loss versus accumulation of saliva as a possible preliminary stage, and diurnal drooling versus nocturnal drooling. In addition, we evaluated the association between drooling severity and the severity of facial and oral motor disorders. We collected age, disease duration, UPDRS III and Hoehn & Yahr stage from 104 consecutive outpatients with PD. Diurnal and nocturnal drooling was evaluated with a validated questionnaire (ROMP-saliva). A speech pathologist, blinded for drooling severity, rated facial expression, involuntary mouth opening and difficulty with nose breathing and also interviewed patients about sleeping position and nose-breathing during the night. Thirty patients (29%) had no complaints with saliva control (‘non-droolers’), 45 patients (43%) experienced accumulation of saliva or only nocturnal drooling (‘pre-droolers’), and 29 (28%) had diurnal drooling (24 of which also drooled during the night; ‘droolers’). The droolers had longer disease duration (10 vs. 7 years, p = 0.01) and drooling was independently associated with involuntary mouth opening (OR = 2.0; 95% CI 1.02–3.99) and swallowing complaints (OR = 1.2; 95% CI 1.03–1.31). Diurnal drooling—defined as dribbling of saliva while awake—is present in about 28% of PD patients. This is less than usually reported. Diurnal drooling typically appeared later in the disease course. The association with oral motor behavior should encourage the development of behavioral treatment approaches

Phenotypic Studies of Natural Killer Cell Subsets in Human Transporter Associated with Antigen Processing Deficiency

Peripheral blood natural killer (NK) cells from patients with transporter associated with antigen processing (TAP) deficiency are hyporesponsive. The mechanism of this defect is unknown, but the phenotype of TAP-deficient NK cells is almost normal. However, we noticed a high percentage of CD56bright cells among total NK cells from two patients. We further investigated TAP-deficient NK cells in these patients and compared them to NK cells from two other TAP-deficient patients with no clinical symptoms and to individuals with chronic inflammatory diseases other than TAP deficiency (chronic lung diseases or vasculitis). Peripheral blood mononuclear cells isolated from venous blood were stained with fluorochrome-conjugated antibodies and the phenotype of NK cells was analyzed by flow cytometry. In addition, 51Chromium release assays were performed to assess the cytotoxic activity of NK cells. In the symptomatic patients, CD56bright NK cells represented 28% and 45%, respectively, of all NK cells (higher than in healthy donors). The patients also displayed a higher percentage of CD56dimCD16− NK cells than controls. Interestingly, this unusual NK cell subtype distribution was not found in the two asymptomatic TAP-deficient cases, but was instead present in several of the other patients. Over-expression of the inhibitory receptor CD94/NKG2A by TAP-deficient NK cells was confirmed and extended to the inhibitory receptor ILT2 (CD85j). These inhibitory receptors were not involved in regulating the cytotoxicity of TAP-deficient NK cells. We conclude that expansion of the CD56bright NK cell subtype in peripheral blood is not a hallmark of TAP deficiency, but can be found in other diseases as well. This might reflect a reaction of the immune system to pathologic conditions. It could be interesting to investigate the relative distribution of NK cell subsets in various respiratory and autoimmune diseases

Patients with primary immunodeficiencies are a reservoir of poliovirus and a risk to polio eradication

ABSTARCT: Immunodeficiency-associated vaccine-derived polioviruses (iVDPVs) have been isolated from primary immunodeficiency (PID) patients exposed to oral poliovirus vaccine (OPV). Patients may excrete poliovirus strains for months or years; the excreted viruses are frequently highly divergent from the parental OPV and have been shown to be as neurovirulent as wild virus. Thus, these patients represent a potential reservoir for transmission of neurovirulent polioviruses in the post-eradication era. In support of WHO recommendations to better estimate the prevalence of poliovirus excreters among PIDs and characterize genetic evolution of these strains, 635 patients including 570 with primary antibody deficiencies and 65 combined immunodeficiencies were studied from 13 OPV-using countries. Two stool samples were collected over 4 days, tested for enterovirus, and the poliovirus positive samples were sequenced. Thirteen patients (2%) excreted polioviruses, most for less than 2 months following identification of infection. Five (0.8%) were classified as iVDPVs (only in combined immunodeficiencies and mostly poliovirus serotype 2). Non-polio enteroviruses were detected in 30 patients (4.7%). Patients with combined immunodeficiencies had increased risk of delayed poliovirus clearance compared to primary antibody deficiencies. Usually, iVDPV was detected in subjects with combined immunodeficiencies in a short period of time after OPV exposure, most for less than 6 months. Surveillance for poliovirus excretion among PID patients should be reinforced until polio eradication is certified and the use of OPV is stopped. Survival rates among PID patients are improving in lower and middle income countries, and iVDPV excreters are identified more frequently. Antivirals or enhanced immunotherapies presently in development represent the only potential means to manage the treatment of prolonged excreters and the risk they present to the polio endgame. Keywords: Poliovirus eradication, Immunodeficiency-associated vaccine-derived polioviruses, Oral poliovirus vaccine, Humoral immunodeficiency, Combined immunodeficiency, Primary immunodeficienc

Controlo químico de infestantes

Uma planta é considerada infestante quando nasce espontaneamente num local e momento indesejados, podendo interferir negativamente com a cultura instalada. As infestantes competem com as culturas para o espaço, a luz, água e nutrientes, podendo atrasar e prejudicar as operações de colheita, depreciar o produto final e assegurarem a reinfestação nas culturas seguintes. Dado o modo de propagação diferenciado das diversas espécies de infestantes, com as anuais a propagarem-se por semente e as perenes ou vivazes a assegurarem a sua propagação através de órgãos vegetativos (rizomas, bolbos, tubérculos, etc.), assim, também o seu controlo quer químico, quer mecânico terá que ser diferenciado, ou seja, para controlar infestantes anuais será suficiente destruir a sua parte aérea, enquanto para controlar infestantes perenes teremos que destruir os seus órgãos reprodutivos. O controlo de infestantes poderá ser químico, através da utilização de herbicidas, ou mecânico pela utilização de alfaias agrícolas, tais como a charrua de aivecas, a charrua de discos, a grade de discos, o escarificador e a fresa. Quando a técnica utilizada na instalação das culturas é a sementeira directa, o controlo das infestantes terá que ser obrigatoriamente químico, enquanto se o recurso à mobilização do solo for a técnica mais utilizada (sistema de mobilização tradicional ou sistema de mobilização reduzida), o controlo das infestantes tanto poderá ser químico como mecânico. Neste trabalho iremos abordar apenas, o controlo químico de infestantes