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.     

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.     

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.     

MUNICIPAL AMALGAMATION AND THE GROWTH OF THE LOCAL PUBLIC SECTOR IN SWEDEN*

ABSTRACT. The proposition that a decentralized structure of local governments can effectively constrain public sector growth is empirically investigated. Data on Swedish municipal sector size for the 1942–87 period are analyzed within the context of a median voter model. The results indicate that decentralization, measured by the number of units of local government serving a given population, constrains the influence of institutional factors (e.g., rent seeking, bureaucracy) on local budgets as long as these units exceed some minimal threshold size. The findings support the view that communities should be given the flexibility to determine their own ideal pattern of local governments.     

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.