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.     

ANALYSIS OF THEORETICAL,METHODOLOGICAL AND EMPIRICAL RESEARCH IN THE JOURNAL OF REGIONAL SCIENCE*

ABSTRACT. This paper examines the impacts of JRS articles in the areas of theory, methods and empirical analysis, as represented by subsequent citations reported in the Social Sciences Citation Index. A model relating the mix of theoretical, methodological and empirical research published in the JRS to subsequent citations is estimated. The time distribution of theoretical and empirical citations also indicates that the knowledge diffusion process in these two areas may be segmented.     

Heterodoxe lehren und ihre widerlegung im Kālacakra-tantra

Summary At the end of the second chapter of the Buddhist Klacakra-Tantra (dated c. 10th century) we find a group of verses in which several doctrines are characterized and criticized: Brahmanism, Vaisnavism, aivism, materialism, Islam and Jainism. Moreover the text refers to Buddhism itself as well as the so-called heterodox Buddhist schools. It is the only old Sanskrit text known until now in which the Islam is mentioned and recognized as a doctrine.     

ROCK PHYSICS AND THE NEOLITHIC AXE TRADE IN GREAT BRITAIN*

This paper is concerned with the products of a number of Neolithic quarries in highland Britain. It investigates the claim that the scale of axe distribution was partly the result of social factors which resulted in these artefacts gaining an added value in areas remote from their sources. One case study considers the sequence of production in Cumbria, in relation to the tensile strength of the rock quarried in different parts of the region. It shows that these considerations had only a limited influence over the choice of stone source and the scale on which it was worked. A second study compares the tensile strength of the main raw materials used for making non-flint axes with the extent to which these sources were actually exploited. Again there is only a limited relationship between the two, suggesting that social factors may indeed have been important.