Between 1992 and 1995, the annual incidence of Creutzfeldt–Jakob disease (CJD) in one of the 96 French départments (adminstrative districts) was found to be about six times higher than the CJD national incidence. Among the 12 definite or probable CJD patients referred during this period within this département, nine originated from a small confined area (30 × 30 km) and seven patients carried the E200K mutation in their prion protein gene (PRNP). Genealogical data showed that these seven cases, together with three other ones previously referred during the 1970–82 period, probably belonged to different branches of the same family which could be traced to the beginning of the eighteenth century. Interestingly enough, all but two patients presented as sporadic cases before the genealogic and genetic studies. To our knowledge, this study is the first describing in France a focal accumulation of CJD associated with the PRNP E200K mutation.

Les encéphalopathies spongiformes subaiguës dues à un agent transmissible non conventionnel (ATNC) sont caractérisées cliniquement par des troubles nerveux sensitifs et moteurs évoluant, après une longue période d’incubation, vers une grande misère physiologique et une issue toujours fatale. Dans les conditions naturelles le principal mode de contagion est la voie orale (aliment souillé, protéines animales non décontaminées...). Le fait qu’une composante génétique intervienne dans la réceptivité des animaux à l’ATNC peut laisser penser à une maladie héréditaire. Ceci conduit l’éleveur à ne pas signaler l’existence de la maladie dans son élevage, lorsque celle-ci n’est pas soumise à déclaration obligatoire.

Invasion of scrapie agent into the central nervous system (CNS) was studied in rats following intracerebral and peripheral inoculation, the latter by injection into intact or transected sciatic nerve. Comparison of sleep-wakefulness alterations, neuropathological features, and time lag of electroencephalographic and clinical signs in the 3 groups suggests that hematogenous spread of infection to the CNS may predominate over neural transport, and that peripheral inoculation may closely approximate natural infection.

A case of Creutzfeldt-Jakob disease (CJD) in a 52-year-old man is described. At post mortem, extensive involvement of white matter was seen. A few similar cases have been reported mainly by Japanese authors.

Discovery oa a second affected branch of a family with transmitted Creutzfeldt-Jakob disease (CJD), originally reported by Buge et al. in 1978, brings the total number of cases to 14 in 3 generations, with at least 20 members of the next young adult generation presently at risk. Complete segregation of the illness to the descendants of these 2 branches, with no skipped generations, and an overall frequency of CJD in affected sibships of 56%, clearly defines a pattern of autosomal dominance. The disease is indifferent to sex, either in terms of affected members (8 males and 6 females) or of lineage (3 fathers and 3 mothers). Acquisition of CJD virus from a point source contamination is unlikely, and case-to-case transmission, if it occurred, would have required an average minimum incubation period of 17 years.

Les caractères cliniques, pathologiques et pathogéniques de la maladie de Creutzfeldt-Jakob sont comparés à ceux de la Tremblante du mouton. Ces maladies sont provoquées par des «virus non conventionnels» transmissibles, qui ont des caractères biologiques semblables. Une étude épidémiologique comparée pourrait apporter des informations permettant d’étayer ou de réfuter l'hypothèse selon laquelle les deux maladies seraient provoquées par le même virus.

Mixed infection of Vero cells with measles and vesicular stomatitis viruses gives rise to phenotypically mixed infections virus particles possessing the properties of pseudotypes. Similar results were obtained when vesicular stomatitis virus was used to superinfect a defective measles system.

The World Health Organization (WHO) convened a Scientific Group to adapt its program in virus diseases to recent progress in virology. The program consists of (a) general activities, such as reference services and the supplying of reagents by the WHO Collaborating Centres and (b) specific activities to solve problems-including the promotion of necessary research-caused by certain diseases of public health importance. The Group reviewed problems caused by influenza and other respiratory viruses, enteroviruses, gastroenteritis viruses (for which types A and B have been proposed as a convenient nomenclature), viral hepatitis, viruses in water and sewage, arboviruses, arenaviruses and Marburg virus, measles and rubella vaccination, smallpox, rabies, chronic infections, herpesviruses, oncogenic viruses, congenital infections, nosocomial infections, chlamydial and rickettsial infections, and mycoplasma infections.