Additional neurological features have recently been described in seven families transmitting pathogenic mutations in OPA1, the most common cause of autosomal dominant optic atrophy. However, the frequency of these syndromal 'dominant optic atrophy plus' variants and the extent of neurological involvement have not been established. In this large multi-centre… (More)
1 Introduction General-purpose commercial disk-based database systems , though widely employed in practice, have failed to meet the performance requirements of applications requiring short, predictable response times, and extremely high throughput rates. Main memory is the only technology capable of these characteristics. DataBlitz is a main-memory storage… (More)
In 1999, the U.S. West Nile (WN) virus epidemic was preceded by widespread reports of avian deaths. In 2000, ArboNET, a cooperative WN virus surveillance system, was implemented to monitor the sentinel epizootic that precedes human infection. This report summarizes 2000 surveillance data, documents widespread virus activity in 2000, and demonstrates the… (More)
Software inspection is an eeective method of defect detection. R e c ent research activity has considered the development of tool support to further increase the eeciency and eeectiveness of inspection, r esulting in a number of prototype t o ols being developed. However, no comprehensive evaluations of these tools have been carried out to determine their… (More)
An outbreak of encephalitis occurred in New York City in late August 1999, the first caused by West Nile virus in North America. Histopathologic and immunopathologic examinations performed on human autopsy materials helped guide subsequent laboratory and epidemiologic investigations that led to identification of the etiologic agent.