Family Clustering of Viliuisk Encephalomyelitis in Traditional and New Geographic Regions

摘要

Viliuisk encephalomyelitis is an acute, often fatal, me-ningoencephalitis that tends to develop into a prolonged chronically progressive panencephalitis. Clinical, neuro-pathologic, and epidemiologic data argue for an infectious cause, although multiple attempts at pathogen isolation have been unsuccessful. To assess mechanisms of disease transmission and spread, we studied 6 multiplex families. Secondary cases occurred among genetically related and unrelated persons in a setting of prolonged intrahousehold contact with a patient manifesting the disease. Transmis-sion to unrelated persons was documented in a densely populated region around the city of Yakutsk in which Viliuisk encephalomyelitis had not been previously known. Initially identifi ed in a small Yakut-Evenk population on the Viliui River of eastern Siberia, the disease subsequently spread through human contacts to new geographic areas, thus characterizing Viliuisk encephalomyelitis as an emerging infectious disease