Viliuisk encephalomyelitis (VE) is a progressive neurodegenerative disorder with a clinical course varying from a relatively acute disease with characteristics of a meningoencephalitis lasting 2 to 6 months to a more prolonged progressive panencephalitic syndrome with a fatal outcome in 1 to 6 years. Some patients develop global dementia and severe spasticity, a residual static condition that may last more than 20 years. For many years, VE was recognized exclusively in a small Middle Viliui region of Siberia. A significant migration from the Viliui valley to more densely populated regions during the 1950s brought VE to settlements in which this disease has previously been unknown. There is strong circumstantial evidence that VE is a communicable disease with a pattern of dissemination characteristic of leprosy and other latent and chronic infectious diseases. The high prevalence and mortality of Ve in some areas, the trend toward further geographic spread, the occurrence of among young people with resulting early incapacitation, combined with the absence of specific therapy, contribute to the seriousness of this emerging neurodegenerative disorder of probably infectious cause.
The specific aim of the proposed collaborative research is to identify the etiologic agent, as well as host-derived disease-specific markers, of VE. Overall, the proposed studies will test the hypothesis that VE is caused by an infectious agent which cannot be cultivated using conventional microbiological techniques. The proposed project will be conducted as a multi-institutional, international, integrated collaboration between investigators at the University of Hawaii at Manoa (UHM), the National Institute of Neurological Disorders and Stroke and the Institute of Health, National Academy of Sciences of the Sakha (Yakut) Republic in Yakutsk, Russia. The applicant component of the project will focus on applying state-of-the-art molecular techniques, to search for non-host- and host- derived mRNA sequences in tissues and biological fluids from VE patients. A complementary approach, consisting of serological tests and consensus sequence-based PCR, will be employed by the collaborating component to identify specific microorganisms of general taxonomic groups which may be etiologically involved in VE. By taking full advantage of he complementary expertise and approaches of the applicant and collaborator, as well as the logistical support of clinical, field and laboratory research consultants, the probably of success should be greatly enhanced. Moreover, the close linkage between the applicant and a world- renowned expert on sensitive molecular technologies for new pathogen discovery will provide unparalleled opportunities for graduate students and post-doctoral fellows at UHM to obtain training in the new and emerging molecular strategies being employed in this collaborative research effort.
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