Family Clustering of Viliuisk Encephalomyelitis in Traditional and New Geographic Regions
Viliuisk encephalomyelitis is an acute, often fatal, meningoencephalitis that tends to develop into a prolonged chronically progressive panencephalitis. Clinical, neuropathologic, and epidemiologic data argue for an infectious cause, although multiple attempts at pathogen isolation have been unsucce...
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| Format: | Text |
| Language: | English |
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| Online Access: | http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.306.3529 http://wwwnc.cdc.gov/eid/article/13/9/pdfs/06-1585.pdf |
| Summary: | Viliuisk encephalomyelitis is an acute, often fatal, meningoencephalitis that tends to develop into a prolonged chronically progressive panencephalitis. Clinical, neuropathologic, 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. Transmission 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. Viliuisk encephalomyelitis (VE) is clinically and pathologically defined as an acute meningoencephalitis that progresses to a more prolonged panencephalitic syndrome (1–3). In a small number of patients, the initial acute phase had gone undetected. Cerebrospinal fluid (CSF) pleocytosis is present during the acute phase and subsequent progressive stages of illness. Postmortem examination identifies diffuse infiltration of the meninges with mononuclear and plasma cells; multiple micronecrotic lesions in the brain |
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