The ocular motor features of adult-onset alexander disease: a case and review of the literature

A 51-year-old Chinese man presented with gaze-evoked nystagmus, impaired smooth pursuit and vestibular ocular reflex cancellation, and saccadic dysmetria, along with a family history suggestive of late-onset autosomal dominant parkinsonism. MRI revealed abnormalities of the medulla and cervical spinal cord typical of adult-onset Alexander disease, and genetic testing showed homozygosity for the p.D295N polymorphic allele in the gene encoding the glial fibrillary acidic protein. A review of the literature shows that ocular signs are frequent in adult-onset Alexander disease, most commonly gaze-evoked nystagmus, pendular nystagmus, and/or oculopalatal myoclonus, and less commonly ptosis, miosis, and saccadic dysmetria. These signs are consistent with the propensity of adult-onset Alexander disease to cause medullary abnormalities on neuroimaging.

Internationaler Zivilprozess 2011, Zusammenspiel des revLugÜ mit dem revSchKG und der schweizerischen ZPO, mit Beiträgen von Prof. Dr. Jürgen Brönnimann, Prof. Dr. Felix Dasser, Prof. Dr. Alexander R. Markus, Prof. Dr. Rodrigo Rodriguez, Prof. Dr. Daniel Staehelin, Prof. Dr. Walter A. Stoffel

Am 10. September 2010 fand in Bern die erste Schweizerische Tagung für Zivilverfahrensrecht des Instituts für Internationales Privatrecht und Verfahrensrecht der Universität Bern statt. Die Tagung mit dem Titel "Internationaler Zivilprozess 2011" befasste sich mit dem Zusammenspiel der am 1.1.2011 in Kraft tretenden neuen oder revidierten Erlasse (ZPO, revLugÜ, und revSchKG) im Rahmen des internationalen Zivilprozesses. Der Tagungsband enthält auf den Tagungsvorträgen basierende Beiträge namhafter Autoren zu aktuellen und praxisrelevanten Themen des neuen internationalen Zivilprozesses, namentlich zum neuen Arrestrecht, zur Behandlung von Zustellungsmängeln unter dem revLugÜ, zum Zahlungsbefehl im Lichte der revLugÜ-Zuständigkeiten, zur vollstreckbaren öffentliche Urkunde sowie zur Rechtshängigkeit und zur Streitgenossenschaft im internationalen Verhältnis. Der Tagungsband "Internationaler Zivilprozess 2011" eröffnet eine neue Schriftenreihe zum Internationalen Privatrecht und Verfahrensrecht.

Perennial Snow and Ice Variations (2000-2008) in the Arctic Circumpolar Land Area from Satellite Observations

Perennial snow and ice (PSI) extent is an important parameter of mountain environments with regard to its involvement in the hydrological cycle and the surface energy budget. We investigated interannual variations of PSI in nine mountain regions of interest (ROI) between 2000 and 2008. For that purpose, a novel MODIS data set processed at the Canada Centre for Remote Sensing at 250 m spatial resolution was utilized. The extent of PSI exhibited significant interannual variations, with coefficients of variation ranging from 5% to 81% depending on the ROI. A strong negative relationship was found between PSI and positive degree-days (threshold 0°C) during the summer months in most ROIs, with linear correlation coefficients (r) being as low as r = −0.90. In the European Alps and Scandinavia, PSI extent was significantly correlated with annual net glacier mass balances, with r = 0.91 and r = 0.85, respectively, suggesting that MODIS-derived PSI extent may be used as an indicator of net glacier mass balances. Validation of PSI extent in two land surface classifications for the years 2000 and 2005, GLC-2000 and Globcover, revealed significant discrepancies of up to 129% for both classifications. With regard to the importance of such classifications for land surface parameterizations in climate and land surface process models, this is a potential source of error to be investigated in future studies. The results presented here provide an interesting insight into variations of PSI in several ROIs and are instrumental for our understanding of sensitive mountain regions in the context of global climate change assessment.

Sensitive and rapid detection of ganciclovir resistance by PCR based MALDI-TOF analysis

Cytomegalovirus (CMV) infection is associated with significant morbidity and mortality in transplant recipients. Resistance against ganciclovir is increasingly observed. According to current guidelines, direct drug resistance testing is not always performed due to high costs and work effort, even when resistance is suspected.

Structural aspects of redox-mediated electron tunneling (Dedicated to the memory of professor Alexander M. Kuznetsov)

We investigated structural aspects of electron transfer (ET) in tunneling junctions (Au(1 1 1)vertical bar FcN vertical bar solution gap vertical bar Au STM tip) with four different redox-active N-thioalk(ano)ylferrocenes (FcN) embedded. The investigated molecules consist of a redox-active ferrocene (Fc) moiety connected via alkyl spacers with N = 4, 6, 8 and 11 carbon atoms to a thiol anchoring group. We found that for short FcNs (N = 4, 6,8) the redox-mediated ET response increases with the increase of the alkyl chain length, while no enhancement of the ET was observed for Fc1 1. The model of two-step ET with partial vibrational relaxation by Kuznetsov and Ulstrup was used to rationalize these results. The theoretical ET steps were assigned to two processes: (1) electron tunneling from the Fc group to the Au tip through the electrolyte layer and (2) electron transport from the Au(1 1 1) substrate to the Fc group through the organic adlayer. We argue that for the three short FcNs, the first process represents the rate-limiting step. The increase of the length of the alkyl chain leads to an approach of the Fc group to the STM tip, and consequently accelerates the first El' step. In case of the Fcl 1 junctions the rather high thickness of the organic layer leads to a decrease of the rate of the second ET step. In consequence, the contribution of the redox-mediated current enhancement to the total tunneling current appears to be insignificant. Our work demonstrates the importance of combined structural and transport approaches for the understanding of Er processes in electrochemical nanosystems. (C) 2010 Elsevier B.V. All rights reserved.