Charcot-Marie-Tooth disease from first description to genetic localization of mutations

Charcot-Marie-Tooth disease is a hereditary motor and sensory neuropathy first described in 1886. Our increasing knowledge of this disease correlates well with the development of methods used in neurology over the past 100 years. Although its physiopathology and treatment is still not fully understood, current developments in techniques are opening the way to future discoveries. We have divided its history into three theoretical periods: the first from 1886 to 1956, which was devoted to clinical and pathological study of the disease; the second from 1956 to 1982, which saw the development of electromyography in the investigation of neuromuscular diseases; and the last and current period based upon genetic research, using the methods of molecular biology.     

CORPORATE IDEOLOGY AND THE POLITICS OF ENTREPRENEURISM IN NEW ORLEANS

In this article, Louis F. Miron presents a case study of the effects of local ideology and culture on corporate-lead progrowth movements in New Orleans. Miron borrows from critical theory to illustrate how "hegemonic" ideologies such as economic growth may be resisted in local settings. The findings suggest that economic structural relations do not predetermine the course of human agency, and that the success of progrowth movements is contingent upon local cultural conditions and ethos. Through a document analysis of "fiscal reform," a pro-growth ideology advanced by corporate and political elites in New Orleans and throughout Louisiana, the author probes the connections among material conditions, ideology, and the politics of entrepreneurism.     

COMPACTNESS AND ELECTORAL BOUNDARY ADJUSTMENT: AN ASSESSMENT OF ALTERNATIVE MEASURES

Spatial compactness has been proposed as a suitable criterion for assessing whether electoral boundaries reflect partisan interests. Although several different measures of spatial compactness have been developed, only limited efforts have been made to compare and evaluate them in the context of electoral redistricting. This paper assesses alternative spatial compactness measures and applies conceptually appealing measures to the provincial ridings proposed by British Columbia's Royal Commission on Electoral Boundaries (1988).     

Rare Himyaritic coins from Hadramawt

Three rare Himyaritic coins found in the territory of the ancient Hadramawt kingdom are published for the first time. Two of the coins bear the name of the king,'Amdan Bayyin, while the third has the name of Tha'ran Ya'ub.     

RARE EARTH ELEMENT DISTRIBUTION PATTERNS IN SHETLAND STEATITE - CONSEQUENCES FOR ARTIFACT PROVENANCING STUDIES

An attempt has been made to relate steatite artifacts to the source of rock from which they were manufactured, using the distribution patterns of rare earth elements. Radiochemical- and instrumental-neutron activation analyses of Shetland steatites suggest that the use of rare earth elements in artifact provenancing may be unreliable. Concentrations of rare earth elements in many Shetland steatites are too low to be measured reliably by a cost effective method. The relative importance of primary igneous and secondary metasomatic processes in controlling steatite rare earth element geochemistry is discussed and geological arguments against the existence of unique rare earth ‘fingerprints’ for steatite are presented.