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Alexander disease (AxD) is a leukodystrophy, described in infantile, juvenile and adult onset forms, due to mutations in the glial fibrillary acid protein (GFAP) gene. Adult-onset AxD (AOAD) has a range of clinical and radiographic phenotypes with the oldest reported onset in the seventh decade.We report a case of AOAD, with onset in the eighth decade, presenting with slow variant orthostatic tremor, which has not been previously described. Genetic analysis revealed a GFAP variant (c.1158CA) that has not been previously reported. Our case serves to expand the diagnostic spectrum of AOAD both clinically and genetically.

In 1984, Watson and Heilman reported a patient with a partial callosal disconnection following an infarction of the anterior portion of her corpus callosum. This woman's performance on line-bisection tasks revealed "callosal disconnection neglect." The objective of this research is to reexamine this woman 34 years after her callosal disconnection to gain information about her recovery. The patient completed visual line-bisection tasks in which horizontal lines were placed in the right, left, and center hemispaces and she performed these bisections using her right or left hand. Unlike her performance 34 years ago in which each hand deviated to its ipsilateral hemispace, with greater deviation when lines were placed in the contralateral rather than ipsilateral hemispace, currently, there were no significant main effects for hand or spatial position. Thus, there were notable differences between this woman's most recent performance on the line bisection and her previous performance 34 years ago. Unlike her prior testing 34 years back, this woman's most recent performance resembled the performance of a previous tested healthy control group for whom differences in hand and hemispace were not found. It remains unclear whether her callosal disconnection neglect improved because each hemisphere learned to allocate ipsilateral spatial attention or because she learned a compensatory strategy in which she turned her body so that the lines placed in her right or left hemispace were now toward her midline.

The terrorist attacks in Paris in 2015 and the highly publicized increase in migration to Israel from France have once again raised the spectre of a permanent Muslim–Jewish antagonism that is thoug...

From the fall of France in 1940 to the Anglo-American liberation of North Africa from Vichy rule (in Morocco and Algeria) and German occupation (in Tunisia), from November 1942 to May 1943, the Second World War profoundly transformed North African youth. For young French-educated Jews living in urban colonial spaces, disillusionment with Vichy France's betrayal of democracy and equality led them to a search for new political models, including Communism.Through the memoirs of three North African Jews that came to politics through local Communist parties, Abraham Serfaty, Daniel Timsit and Gilbert Naccache, Communism in its wartime form (allied with Western democracies) provided an ideal mediating force. For a generation that wanted to fight against the anti-Jewish measures of the Vichy and Nazi regimes, as well as to abolish colonial distinctions between indigenous Jews and Muslims and Europeans, Communism proposed radical equality in a supra-national framework. As the authors/activists look back on the fa...

Born to a Jewish family in Tunisia in 1950 near the end of the French Protectorate, Colette Fellous grew up among multiple worlds. Educated entirely in French, her parents passed on only fragments of a disappearing Judeo-Arab past. Her autobiographical works return incessantly to the family's apartment and daily life, focusing on everyday objects which bridge family history and History writ large. This article analyzes her relationship with the most frequently recurring of these objects, a reproduction of Paul Cezanne's Card Players that hung in the middle of the Fellous home. Through a complex play of verbal and visual, Fellous constantly destabilizes the traditional ekphrastic scenario through an emphasis on silence, fragmentation, and chance. Imagining the Card Players as surrogate parents in a colonial family romance, she uses card playing itself as a metaphor for memory. Hence, the relationship between author, reader, and image becomes multi-layered, with each partner in the game continually drawing ...

This article examines the construction of memory through relation to lost Jewish places and spaces in Algeria in recent French-language autobiographical writing of Albert Bensoussan, Alice Cherki, Annie Cohen, Jean Cohen, Attica Guedj, Benjamin Stora and Daniel Timsit. Published between 1995 and 2010, these works represent a duel interrogation of memory through evocation of place: first, remembering the ways Jews carved out space for themselves at home, at school, in spaces of leisure and sacred spaces, second, where Jews situate themselves given the lack of Jewish presence in Algeria today, and the ways the community gets forgotten in its present-day exile in France and Israel. Jewish writers retrospectively relocate themselves to Algeria in an effort to maintain their position in between Algerian Muslims and pieds-noirs, forever attached to both, but reducible to neither.

(2010). An Integrated Look at Prejudice. Contemporary Psychoanalysis: Vol. 46, No. 4, pp. 589-593.

Lifeline Australia Inc. provides a free 24-hour telephone counselling and referral service to all Australians. The trained telephone counsellors of the service record information on many of their c...

To examine the structural determinants necessary for TC10 trafficking, localization, and function in adipocytes, we generated a series of point mutations in the carboxyl-terminal targeting domain of TC10. Wild-type TC10 (TC10/WT) localized to secretory membrane compartments and caveolin-positive lipid raft microdomains at the plasma membrane. Expression of a TC10/C206S point mutant resulted in a trafficking and localization pattern that was indistinguishable from that of TC10/WT. In contrast, although TC10/C209S or the double TC10/C206,209S mutant was plasma membrane localized, it was excluded from both the secretory membrane system and the lipid raft compartments. Surprisingly, inhibition of Golgi membrane transport with brefeldin A did not prevent plasma membrane localization of TC10 or H-Ras. Moreover, inhibition of trans-Golgi network exit with a 19 degrees C temperature block did not prevent the trafficking of TC10 or H-Ras to the plasma membrane. These data demonstrate that TC10 and H-Ras can both traffic to the plasma membrane by at least two distinct transport mechanisms in adipocytes, one dependent upon intracellular membrane transport and another independent of the classical secretory membrane system. Moreover, the transport through the secretory pathway is necessary for the localization of TC10 to lipid raft microdomains at the plasma membrane.

First ladies have overseen historic renovations to the Executive Mansion, stumped long and hard on the campaign trail, edited presidential speeches, advised on cabinet appointments, and championed ...

Numerous polls have offered rankings of the presidents but little effort has been made to rate the performance of the president's spouse. Not only has there been far less academic attention devoted to the first ladies, but there are difficulties inherent in assessing this “office” that presidential scholars need not worry about when ranking the commanders-in-chief. Yet, the first lady has emerged as a political “institution” worthy of scholarly attention, including an attempt at ranking them. This article provides the results of a poll ranking the first ladies, along with an analysis of the rankings between activist and traditionalist first ladies, first ladies of different historical periods, and between the presidents and their spouses.

(1998). Incorporating the First Ladies into the Classroom. The Social Studies: Vol. 89, No. 4, pp. 165-170.

The question of the nature of the relationship between U.S. foreign policy and international human rights remains unanswered. While research exists, there is little consensus on the matter. Moreover, most of the research is limited in scope and methodology to case studies, analyses of a single year, or focusses solely on economic aid allocations as a proxy for U.S. foreign policy. This study comprehensively conceptualizes U.S. foreign policy by analyzing U.S. trade, investment, military arms sales, and various categories of foreign aid with the human rights practices of recipient nations in Latin America over a three year period. It was found that human rights tended not to be a consideration in allocating foreign aid and that it was linked to foreign investments and arms sales during only one of the three years studied. However, a strong relationship existed between human rights and bilateral trade with Latin America, in that the United States traded with nations having the best human rights records.

Several types of porous media heat exchangers are being evaluated for use infusion applications. Broadly, these devices can be classified as capillary-pumped (heat pipes) or mechanically-pumped hea...

Thermal response and thermal fatigue tests of four 5 mm thick beryllium tiles on a Russian divertor mock-up were completed on the Electron Beam Test System at Sandia National Laboratories. The beryllium tiles were diffusion bonded onto an OFHC copper saddleblock and a DSCu (MAGT) tube containing a porous coating. Thermal response tests were performed on the tiles to an absorbed heat flux of 5 MW/m{sup 2} and surface temperatures near 300{degrees}C using 1.4 MPa water at 5.0 m/s flow velocity and an inlet temperature of 8-15{degrees}C. One tile was exposed to incrementally increasing heat fluxes up to 9.5 MW/m{sup 2} and surface temperatures up to 690{degrees}C before debonding at 10 MW/m{sup 2}. A third tile debonded after 9200 thermal fatigue cycles at 5 MW/m{sup 2}, while another debonded after 6800 cycles. In all cases, fatigue failure occurred in the intermetallic layers between the beryllium and copper. No fatigue cracking of the bulk beryllium was observed. During thermal cycling, a gradual loss of porous coating produced increasing sample temperatures. These experiments indicate that diffusion-bonded beryllium tiles can survive several thousand thermal cycles under ITER relevant conditions without failure. However, the reliability of the diffusion bonded Joint remains a serious issue.

(1996). Second International Energy Agency Workshop on Beryllium Technology for Fusion, Jackson Lake Lodge, Wyoming, September 6–8, 1995. Fusion Technology: Vol. 29, No. 3, pp. 409-412.

A modified procedure for the title transformation is described which avoids: (i) a potentially hazardous borane reduction step, and (ii) the intermediacy of water soluble amino alcohols.

We have defined neglect as a failure to report, respond, or orient to novel or meaningful stimuli presented on the side opposite a brain lesion that cannot be attributed to either elemental sensory or motor deficits (Heilman, Watson, & Valenstein, 1993). Many subtypes of neglect have been described, distinguished by the distribution of abnormal behaviour (e.g. spatial versus personal neglect) and the means by which the abnormal behaviour is elicited (e.g. inattention versus extinction). Although at one time we thought that the variety of neglect subtypes could be explained by variations in severity and in means of elicitation, we currently believe that neglect is not a unitary disorder. There is increasing evidence that neglect can be subdivided by the presumed underlying mechanisms: inattention (sensory neglect), disorders of action and intention (motor neglect), and representational disorders.

This paper examines empirically the influences on managerial remuneration in a sample of 97 UK small and medium-sized enterprises (SMEs). The empirical analysis, based on data obtained from intervi...

This paper examines the UK system of corporate governance, the remuneration of senior executives and the Cadbury Committee's proposals for increasing managerial accountability to shareholders. We argue that the UK governance system does not provide shareholders with incentives to seek ‘voice’ solutions to problems of corporate governance and that the Cadbury proposals, which rely on market-based regulation, will do little to alter this. The design and monitoring of executive remuneration schemes is costly and, because of lack of transparency, it is unclear that these performance related schemes are necessarily in shareholders' interests. The empirical evidence relating to the role of non-executive directors and remuneration committees, as advocated by the Cadbury report, also suggests that this process is largely under the control of the executive directors themselves.

Tokamaks currently in operation deposit on the order of 1-30 MW/m2 onto plasma facing surfaces during normal operation and hundreds of MW/m2 for shorter periods of time (0.1–3 ms) during disruptions. Disruption deposited energies on future high-power tokamaks may be well in excess of 20 MJ/m2 Design of plasma facing components (PFCs) for such severe environments requires considerable advancements in materials development, armor tile bonding to actively cooled substrates, heat transfer, and many other areas of engineering concern. Considerable improvements in PFC performance, reliability and lifetime can also be accomplished through improved understanding and control of the edge plasma boundary layer. This paper covers both engineering and edge plasma physics issues that must be addressed in the development of reliable PFCs for ITER. Several specific examples are addressed since a complete treatment of all critical development issues would be lengthy. Topics covered include impurity generation and ...

The divertor of a near-term fusion device has to withstand high heat fluxes, heat shocks, and erosion caused by the plasma. Furthermore, it has to be maintainable through remote techniques. Above a...

The monoblock geometry is proposed for the ITER Physics Phase divertor for brazing of carbon armor tiles to copper or molybdenum cooling tubes. Elastic/plastic finite element analyses predicted high residual stresses except with OFHC copper. Samples of pyrolytic graphite tiles brazed to OFHC copper, Glidcop™ Al-15 copper alloy, and molybdenum tubing show cracking in all of the samples, except with the OFHC copper. A 3-tile divertor target consisting of 12 mm thick pyrolytic graphite brazed with a copper-silver alloy to a 12 mm diameter OFHC copper tubing was tested at 15 MW/m2 with a rastered 30 keV electron beam for 1000 thermal cycles. A gradual rise in surface temperature from 1000 C to 1200 C over the 1000 cycles was observed, along with hot stripes (1500 C) at the tile edges. However, no delamination cracks could be detected.

Critical Heat Flux (CHF), also called burnout, is one of the major design limits for water-cooled divertors in tokamaks. Another important design issue is the correct thermal modeling of the divert...

Whether it was the Christian crusades of the past or Islamic jihad today, religion has been a central part of the clash of cultures and civilizations to the extent that one wonders if it is possibl...

The results indicate that although interest rate premia reflect a number of small firm risk characteristics, they do not seem to have significantly benefited from the increased bank competition of the late 1980s.

In recent years there has been much debate in the UK regarding the value and feasibility of the small company audit. The lack of formal internal control systems and the application of universal auditing standards has given rise to the use of a special ‘Small Company (Example 6) Audit Qualification’. However, doubts concerning the uniformity of practice between auditors has created difficulties for users in interpreting its meaning. This paper examines the extent to which a number of financial, organisational and auditor variables are able to explain the receipt of a ‘Small Company Audit Qualification’. The main empirical findings, using multivariate logistic analysis on a sample of 540 small company reports, are that companies audited by large audit practices, companies which had a prior year qualification, a secured loan, declining earnings, large audit lags and few non-director shareholders were more likely to receive an audit qualification than other companies.

Information concerning past delays in submitting financial statements to Companies House has been found to be an important variable in predicting small company financial failure. However, a distinctive feature of the small company reporting environment is that a large proportion of small companies approaching financial failure fail to submit any accounts in the year(s) immediately prior to failure. This paper presents evidence suggesting that non-submission information can usefully be incorporated into a failure prediction model along with information concerning finite delays in reporting and conventional financial ratios.

(1989). THE NATURAL RESOURCES DEFENSE COUNCIL/USER ACADEMY OF SCIENCES JOINT PROGRAM ON ENERGY CONSERVATION. Soviet Geography: Vol. 30, No. 10, pp. 730-735.

A collaborative program is underway to field a comprehensive pump limiter experimental program on TEXTOR: Advanced Limiter Test-I (ALT-I). Either of two interchangeable limiter modules may be attached to an insertion/rotation mechanism to direct particles to the about1 m/sup 3/ pumping chamber. Pumping is provided primarily by a solid getter assembly at about20,000 1/sec. Variation of geometric dimensions and gas puffing in the modules will allow the study of plasma and neutral interaction in differing recycle regimes. Multiple diagnostic systems are incorporated into the pump limiter design for use in conjunction with TEXTOR plasma diagnostics. Initial experiments are scheduled for December 1983.

The Fraunhofer line discriminator (FLD), an airborne electro-optical instrument designed to measure solar-stimulated luminescence, has a sensitivity approaching that of current laboratory fluorescence spectrometers. The feasibility of using an airborne FLD for detection of near-coastal and open-ocean phytoplankton was established by using a laboratory fluorescence spectrometer to measure the luminescence of phytoplankton and to determine the minimum concentration detectable with an FLD Laboratory measurements of the excitation spectra of 13 species of phytoplankton (six diatoms, five dinoflagellates and two chrysophytes) were obtained with the emission wavelength held constant at 656.3 nm and the excitation wavelength scanned from 320 to 640 nm. Integrated excitation intensities were normalized to a standard concentration of rhodamine wt dye and the resulting luminescence compared to the minimum detectable FLD level of 0.12 parts per billion (p.p.b.) rhodamine wt. Results demonstrated that all 13...

Advanced Limiter Test II (ALT-II) is an axisymmetric toroidal belt pump limiter scheduled for installation on the TEXTOR tokamak in 1986. ALT-II consists of eight blade segments mounted on adjustab...

Sandia National Laboratories, Division of Fusion Technology, has performed a series of tests in support of the Compact Ignition Tokamak first-wall tile design. A screening study was done to rank the thermal shock resistance of 25 candidate graphite materials. Standardized thermal shock samples were subjected to identical intense heat pulses generated by the electron beam test system. Most of the fine-grained graphites cracked, but none of the carbon-carbon (C-C) composite samples fractured. The best performing fine-grained graphites were, in order, Union Carbide's TS-1909, TS-1792, ATJ-S, and CGW, as well as Fiber Material Inc.'s (FMI's) high-density graphite (HDG).