Intravenous immunoglobulin in acute Sydenham's chorea: A systematic review.

Abstract Sydenham's chorea (SC) is a major manifestation seen in 25% of patients with acute rheumatic fever. SC is the prototypic autoimmune neurological disorder, which has a less appreciated associated risk of psychiatric morbidity. We undertook a systematic review to examine whether the use of intravenous immunoglobulin affects clinical recovery and morbidity

Imaging of the urinary tract: the role of CT and MRI

Computed tomography (CT) and magnetic resonance imaging (MRI) are increasingly valuable tools for assessing the urinary tract in adults and children. However, their imaging capabilities, while overlapping in some respects, should be considered as complementary, as each technique offers specific advantages and disadvantages both in actual inherent qualities of the technique and in specific patients and with a specific diagnostic question. The use of CT and MRI should therefore be tailored to the patient and the clinical question. For the scope of this article, the advantages and disadvantages of these techniques in children will be considered; different considerations will apply in adult practice

Archaeology and Desertification in the Wadi Faynan: the Fourth (1999) Season of the Wadi Faynan Landscape Survey

Reproduced with permission of the publisher. © 2000 Council for British Research in the Levant. Details of the publication are available at: http://www.cbrl.org.uk/Publications/publications_default.shtmThis report describes the fourth season of fieldwork by an interdisciplinary team of archaeologists and geographers working together to reconstruct the landscape history of the Wadi Faynan in southern Jordan. The particular focus of the project is the long-term history of inter-relationships between landscape and people, as a contribution to the study of processes of desertification and environmental degradation. The 1999 fieldwork contributed significantly towards the five Objectives defined for the final two field seasons of the project in 1999 and 2000: to map the archaeology outside the ancient field systems flooring the wadi that have formed the principal focus of the archaeological survey in the previous seasons; to use ethnoarchaeological studies both to reconstruct modern and recent land use and also to yield archaeological signatures of land use to inform the analysis of the survey data; to complete the survey of ancient field systems and refine understanding of when and how they functioned; to complete the programme of geomorphological and palaeoecological fieldwork, and in particular to refine the chronology of climatic change and human impacts; and to complete the recording and classification of finds

Getting shot of elves: healing, witchcraft and fairies in the Scottish witchcraft trials

This paper re-examines the evidence of the Scottish witchcraft trials for beliefs associated by scholars with "elf-shot." Some supposed evidence for elf-shot is dismissed, but other material illuminates the interplay between illness, healing and fairy-lore in early modern Scotland, and the relationship of these beliefs to witchcraft itself

Phenotypic insights into ADCY5-associated disease

BACKGROUND: Adenylyl cyclase 5 (ADCY5) mutations is associated with heterogenous syndromes: familial dyskinesia and facial myokymia; paroxysmal chorea and dystonia; autosomal-dominant chorea and dystonia; and benign hereditary chorea. We provide detailed clinical data on 7 patients from six new kindreds with mutations in the ADCY5 gene, in order to expand and define the phenotypic spectrum of ADCY5 mutations. METHODS: In 5 of the 7 patients, followed over a period of 9 to 32 years, ADCY5 was sequenced by Sanger sequencing. The other 2 unrelated patients participated in studies for undiagnosed pediatric hyperkinetic movement disorders and underwent whole-exome sequencing. RESULTS: Five patients had the previously reported p.R418W ADCY5 mutation; we also identified two novel mutations at p.R418G and p.R418Q. All patients presented with motor milestone delay, infantile-onset action-induced generalized choreoathetosis, dystonia, or myoclonus, with episodic exacerbations during drowsiness being a characteristic feature. Axial hypotonia, impaired upward saccades, and intellectual disability were variable features. The p.R418G and p.R418Q mutation patients had a milder phenotype. Six of seven patients had mild functional gain with clonazepam or clobazam. One patient had bilateral globus pallidal DBS at the age of 33 with marked reduction in dyskinesia, which resulted in mild functional improvement. CONCLUSION: We further delineate the clinical features of ADCY5 gene mutations and illustrate its wide phenotypic expression. We describe mild improvement after treatment with clonazepam, clobazam, and bilateral pallidal DBS. ADCY5-associated dyskinesia may be under-recognized, and its diagnosis has important prognostic, genetic, and therapeutic implications. © 2016 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society

Precision overhead irrigation is suitable for several Central Valley crops

Overhead systems are the dominant irrigation technology in many parts of the world, but they are not widely used in California even though they have higher water application efficiency than furrow irrigation systems and lower labor requirements than drip systems. With water and labor perennial concerns in California, the suitability of overhead systems merits consideration. From 2008 through 2013, in studies near Five Points, California, we evaluated overhead irrigation for wheat, corn, cotton, tomato, onion and broccoli as an alternative to furrow and drip irrigation. With the exception of tomato, equal or increased yields were achieved with overhead irrigation. Many variables are involved in the choice of an irrigation system, but our results suggest that, with more research to support best management practices, overhead irrigation may be useful to a wider set of California farmers than currently use it

Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination.

Abstract OBJECTIVE: We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. METHODS: We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients. RESULTS: The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. ON was the dominant phenotype (UON 35%, BON 19%) of all clinical episodes. 109/226 (48%) MRIs had no brain lesions. Patients were steroid responsive, but 70% of episodes treated with oral prednisone relapsed, particularly at doses <10\u2009mg daily or within 2 months of cessation. Immunotherapy, including maintenance prednisone (P=0.0004), intravenous immunoglobulin, rituximab and mycophenolate, all reduced median ARRs on-treatment. Treatment failure rates were lower in patients on maintenance steroids (5%) compared with non-steroidal maintenance immunotherapy (38%) (P=0.016). 58% of patients experienced residual disability (average follow-up 61 months, visual loss in 24%). Patients with ON were less likely to have sustained disability defined by a final EDSS of 652 (OR 0.15, P=0.032), while those who had any myelitis were more likely to have sustained residual deficits (OR 3.56, P=0.077). CONCLUSION: Relapsing MOG antibody-associated demyelination is strongly associated with ON across all age groups and ADEM in children. Patients are highly responsive to steroids, but vulnerable to relapse on steroid reduction and cessation

Ten Misconceptions from the History of Analysis and Their Debunking

The widespread idea that infinitesimals were "eliminated" by the "great triumvirate" of Cantor, Dedekind, and Weierstrass is refuted by an uninterrupted chain of work on infinitesimal-enriched number systems. The elimination claim is an oversimplification created by triumvirate followers, who tend to view the history of analysis as a pre-ordained march toward the radiant future of Weierstrassian epsilontics. In the present text, we document distortions of the history of analysis stemming from the triumvirate ideology of ontological minimalism, which identified the continuum with a single number system. Such anachronistic distortions characterize the received interpretation of Stevin, Leibniz, d'Alembert, Cauchy, and others.Comment: 46 pages, 4 figures; Foundations of Science (2012). arXiv admin note: text overlap with arXiv:1108.2885 and arXiv:1110.545

Volcanic impacts on the Holocene vegetation history of Britain and Ireland? A review and meta-analysis of the pollen evidence

Volcanic ash layers show that the products of Icelandic volcanism reached Britain and Ireland many times during the Holocene. Historical records suggest that at least one eruption, that of Laki in a.d. 1783, was associated with impacts on vegetation. These results raise the question: did Icelandic volcanism affect the Holocene vegetation history of Britain and Ireland? Several studies have used pollen data to address this issue but no clear consensus has been reached. We re-analyse the palynological data using constrained ordination with various representations of potential volcanic impacts. We find that the palynological evidence for volcanic impacts on vegetation is weak but suggest that this is a case of absence of evidence and is not necessarily evidence of absence of impact. To increase the chances of identifying volcanic impacts, future studies need to maximise temporal resolution, replicate results, and investigate a greater number of tephras in a broader range of locations, including more studies from lake sediments

Volcanic Gases:Silent Killers

This is the accepted manuscript. The final version is available at http://link.springer.com/chapter/10.1007%2F11157_2015_14.Volcanic gases are insidious and often overlooked hazards. The effects of volcanic gases on life may be direct, such as asphyxiation, respiratory diseases and skin burns; or indirect, e.g. regional famine caused by the cooling that results from the presence of sulfate aerosols injected into the stratosphere during explosive eruptions. Although accounting for fewer fatalities overall than some other forms of volcanic hazards, history has shown that volcanic gases are implicated frequently in small-scale fatal events in diverse volcanic and geothermal regions. In order to mitigate risks due to volcanic gases, we must identify the challenges. The first relates to the difficulty of monitoring and hazard communication: gas concentrations may be elevated over large areas and may change rapidly with time. Developing alert and early warning systems that will be communicated in a timely fashion to the population is logistically difficult. The second challenge focuses on education and understanding risk. An effective response to warnings requires an educated population and a balanced weighing of conflicting cultural beliefs or economic interests with risk. In the case of gas hazards, this may also mean having the correct personal protection equipment, knowing where to go in case of evacuation and being aware of increased risk under certain sets of meteorological conditions. In this chapter we review several classes of gas hazard, the risks associated with them, potential risk mitigation strategies and ways of communicating risk. We discuss carbon dioxide flows and accumulations, including lake overturn events which have accounted for the greatest number of direct fatalities, the hazards arising from the injection of sulfate aerosol into the troposphere and into the stratosphere. A significant hazard facing the UK and northern Europe is a “Laki”-style eruption in Iceland, which will be associated with increased risk of respiratory illness and mortality due to poor air quality when gases and aerosols are dispersed over Europe. We discuss strategies for preparing for a future Laki style event and implications for society