Therapy resistant neonatal seizures, linear vesicular rash, and unusually early neuroradiological changes: incontinentia pigmenti : A case report, literature review and insight into pathogenesis

CASE PRESENTATION: A substance abusing G2P1 mother spontaneously delivered at term an appropriate for gestational age girl. Neonatal seizures appeared at 21 hours and empiric anticonvulsive and antimicrobial treatment was started. At 25 hours, first vesicles appeared. While routine evaluations remained normal, a head CT revealed multifocal ischemic injuries, and a later MRI showed multifocal petechiae and diffusion abnormalities in the corticospinal tracts. The clinical diagnosis of incontinentia pigmenti (stage 1) was secured by histopathology. Follow-up at 13 months showed global developmental delay. DISCUSSION: We discuss the unusually early bilateral, fronto-occipital corticomedullar ischemias (CT day 3). On the MR imaging (day 7) extensive symmetric cerebral corticomedullar destruction and diffusion sequences with corticospinal tracts abnormalities are seen, which then evolve (day 26) to extensive symmetric cerebral destruction. We review the literature, genetics, suspected pathophysiology and possible neonatal manifestation. CONCLUSION: Incontinentia pigmenti is rare and, therefore, diagnosis is frequently delayed. Nevertheless, in the setting of therapy refractory seizures, excluded infections, and linear vesicular rash, a high index of suspicion is needed. This is the first report of simultaneous corticomedullar involvement as early as the third day of life.

Impulses for an Effective and Modern Data Protection System

A substantial reform of data protection law is on the agenda of the European Commission as it is widely agreed that data protection law is faced by lots of challenges, due to fundamental technical and social changes or even revolutions. Therefore, the authors have issued draft new provisions on data protection law that would work in both Germany and Europe. The draft is intended to provide a new approach and deal with the consequences of such an approach. This article contains some key theses on the main legislatory changes that appear both necessary and adequate.

Recommendations for the use of ultrasound in rheumatoid arthritis: literature review and SONAR score experience

Ultrasound (US) has become a useful tool in the detection of early disease, differential diagnosis, guidance of treatment decisions and treatment monitoring of rheumatoid arthritis (RA). In 2008, the Swiss Sonography in Arthritis and Rheumatism (SONAR) group was established to promote the use of US in inflammatory arthritis in clinical practice. A scoring system was developed and taught to a large number of Swiss rheumatologists who already contributed to the Swiss Clinical Quality Management (SCQM) database, a national patient register. This paper intends to give a Swiss consensus about best clinical practice recommendations for the use of US in RA on the basis of the current literature knowledge and experience with the Swiss SONAR score. Literature research was performed to collect data on current evidence. The results were discussed among specialists of the Swiss university centres and private practice, following a structured procedure. Musculoskelatal US was found to be very helpful in establishing the diagnosis and monitoring the evolution of RA, and to be a reliable tool if used by experienced examiners. It influences treatment decisions such as continuing, intensifying or stepping down therapy. The definite modalities of integrating US into the diagnosis and monitoring of RA treatments will be defined within a few years. There are, however, strong arguments to use US findings as of today in daily clinical care. Some practical recommendations about the use of US in RA, focusing on the diagnosis and the use of the SONAR score, are proposed.

Correlating the Ancient Maya and Modern European Calendars with High-Precision AMS 14C Dating

The reasons for the development and collapse of Maya civilization remain controversial and historical events carved on stone monuments throughout this region provide a remarkable source of data about the rise and fall of these complex polities. Use of these records depends on correlating the Maya and European calendars so that they can be compared with climate and environmental datasets. Correlation constants can vary up to 1000 years and remain controversial. We report a series of high-resolution AMS C-14 dates on a wooden lintel collected from the Classic Period city of Tikal bearing Maya calendar dates. The radiocarbon dates were calibrated using a Bayesian statistical model and indicate that the dates were carved on the lintel between AD 658-696. This strongly supports the Goodman-Martinez-Thompson (GMT) correlation and the hypothesis that climate change played an important role in the development and demise of this complex civilization.

River loads and modern denudation of the Alps — A review

This paper presents the first comprehensive analysis of sediment and dissolved load across an entire mountain range. We investigate patterns and rates of modern denudation of the European Alps based on a compilation of data about river loads and reservoir sedimentation from 202 drainage basins that are between ca. 1 to 10,000 km2 large. The study basins cover about 50% of the total area of the Alps. Modern glaciated basins have the highest sediment yields of up to 7000 t km− 2 a− 1, which are on average 5 to 10 times higher than in non-glaciated basins. Likewise sediment yield and glacial cover are positively correlated. Instead, relief is a relatively weak predictor of sediment yield. The strong glacial impact in the correlations is due to glacier recession since the 19th century as well as due to glacial conditioning during repeated Quaternary glaciations which have produced the strong transient state of the Alpine landscape. We suggest that this is the major cause for ca. 3 fold enhanced denudation of the western compared to the eastern Alps. Chemical denudation rates are highest in the external Alps dominated by carbonate sedimentary rocks, where they make up about one third of total denudation. The high rates cannot be explained without anhydrite dissolution. We estimated that only 45% of the sediments mobilized in headwaters are exported out off the Alps, most sediments being trapped in artificial reservoirs. The total amount of sediment annually trapped within the Alps equates to 43 Mt. When corrected for sediment storage, we obtain an area-weighted mean total denudation rate for the Alps of about 0.32 mm a− 1. The pre-dam rate might be as high as 0.42 mm a− 1. In total, ca. 35 plus 23 Mt of mass are exported each year out of the Alps as solids and solutes, respectively. These rates are not enough to out pace modern rock uplift. Nevertheless, pattern of sediment yield across the Alps coincides roughly with the intensity of glacial conditioning and modern rock uplift, supporting the hypothesis of an erosion-driven uplift of the Alps.

The Jewish law of marriage and divorce in ancient and modern times and its relation to the law of the State

by M. Mielziner

Cantorial anthology of traditional and modern synagogue music : arranged for cantor and choir with organ accompaniment

comp. and ed. by Gershon Ephros