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.     

Culture contact,continuity, and change on the Gold Coast,AD 1400–1900

This article briefly examines the consequences of the European presence on the Gold Coast, focusing on archaeological, ethnographic, and historical data from the African settlement of Elmina, Ghana. Documentary sources indicate that there was extensive change in sociopolitical institutions, economic relations, and other aspects of Gold Coast society in the centuries following the advent of European trade in the late fifteenth century. Archaeological survey and excavation of some 30 structures at Elmina similarly indicates a great deal of change in construction technology and material culture during the post-European contact period. However, examination of artefact patterning and associations indicates that in certain respects there was little change in terms of people's shared world view and belief systems. This picture can be contrasted with data from sites in other areas which may have experienced a great deal of culture change during the post-European contact period.

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Résumé Cet article examine brièvement les conséquences de la présence européenne sur la Côte de l'Or, se penchant plus particulièrement sur les données archéologiques, ethnographiques et historiques du site d'Elmina au Ghana. Les sources documentaries indiquent qu'un changement de grande envergure affectant les institutions socio-politiques, les relations économiques et d'autres aspects de la société de la Côte de l'Or pris place durant les siècles suivant l'arrivée du commerce européen à la fin du quinzième siècle. Un survey archéologique et les fouilles d'environ 30 structures à Elmina, indiquent aussi qu'un changement étendu des techniques de construction et de la culture matérielle s'effectue après la période de contact européen. Toutefois, l'étude de la distribution et des associations des objets, indique que le système de croyance et l'idée du monde partagée par cette société furent peu affectés.Cette image peut être comparée avec les données obtenues sur d'autres sites ayant fait l'expérience d'un changement culturel important pendant la période postérieure aux contacts européens.

     

Neutron activation analysis of stone from the Chadron Formation and a Clovis Site on the Great Plains

Cryptocrystalline silicates from the Chadron Formation were widely used by prehistoric human groups in the Great Plains of North America. There are two documented quarry areas: Flattop Butte in Colorado and the White River Badlands of South Dakota. Cryptocrystalline silicates from these sources are visually indistinguishable, making it difficult to determine the sources of artefacts found at archaeological sites. In this investigation, neutron activation analysis is used to distinguish the two sources. This technique also identifies lithic material from the Clovis-age Eckles site in Kansas as being from Flattop Butte.     

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.     

ANALYSE ET CARTOGRAPHIE DES DOMMAGES DU SÉISME SURVENU AU QUÉBEC LE 25 NOVEMBRE 1988

Dans le cadre d'un projet de recherche en cours portant sur l'évaluation de la vulnérabilité municipale aux catastrophes naturelles (Boivin 1990), un examen des dommages du séisme du 25 novembre 1988 a été mené afin de mieux documenter l'analyse du risque séismique au Québec. À partir de sources variées de rapports de dommages mais, plus spécifiquement, par l'étude des demandes liées au programme de dédommagement mis sur pied par le gouvernement du Québec, une analyse et une cartographie des dommages rapportés est fournie. Les résultats montrent que la majorité des dommages sont concentrés près de l'épicentre dans les régions du Saguenay/Lac Saint-Jean et de Québec. Les dégats répertoriés concernent principalement les ouvertures (portes et (enê tres) avec 11%, les murs intérieurs ou extérieurs (29%), l'apparition de fissures (72%), les fondations (44%) et les cheminées (29%). Au total, 1521 cas de dommages ont été rapportés et analysés dans ce projet de recherche.