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Diagnostic criteria

Viliuisk Encephalomyelitis – an Emerging Infectious Disease in Eastern Siberia L. Goldfarb, A. Chepurnov, V. Vladimirtsev, V.Osakovskiy, T.Sivtseva, K Master, V.Alexeev and D.C Gajdusek,.

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Diagnostic criteria

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  1. Viliuisk Encephalomyelitis – an Emerging Infectious Disease in Eastern SiberiaL. Goldfarb, A. Chepurnov, V. Vladimirtsev, V.Osakovskiy, T.Sivtseva, K Master, V.Alexeev and D.C Gajdusek,

  2. Vilyuisk encephalomyelitis (VE) is one of the most mistery geographical diseases in the world. It is similar to such wellknown slow infections of nervous system as kuru, Creutzefeldt-Jakob disease, leukoencephalopathy, and amiotrophical leukospongios. VE is a unique 100% fatal disorder with distinct features of a slow virus disease. VE are known as a disease amazing the representatives of radical nationalities of republic Sacha (Yakutia) - of the Yakuts, ewens and ewenks, and till now it is still the reason of premature death 1,5 % of the population of republic. Investigations performed in 1970-1988 by Institute of Polyomyelitis and Viral Encephalitis of former Soviet Union excluded VE from ethnic hereditary diseases and allowed supposing the leading role of viral factors in the illness etiology. However, the agent of VE has not been isolated yet despite numerous attempts since 1954. Severity of the central nervous system injury, which leads to the significant loss of physical and mental abilities and to the death find the reflection in the pathogistologikal picture.

  3. VE is a heavy disease of the man with a clinical picture of sharp meningoencefalitis in an early stage of illness, which passing in progressing chronic process with development dementia and various movement disorders. The illness invariable comes to an end by death in terms from several months about several years. VE is the infection with slow spread from traditional areas in Vilyuisk region to new territories with dense population. The current state of VE situation requires the deep investigations directed to the revealing of etiologic agent of the disease, epidemiological ways, virological, immunological, and pathohistilogical studies. And certainly major task is revealing the infectious agent.

  4. Diagnostic criteria 1. Acute VE • Prolonged fever (more than 7 days), Chills, Severe headache • Confusion, Coma, Delirium • Meningo-encephalitis with CSF inflammatory change • Pyramidal signs • Extrapyramidal signs • Rapidly progredient fatal illness (typical duration - several months) • Neuropathology: Inflamed meninges, Randomly scattered multiple small round necrotic areas, and Perivascular infiltrates

  5. Diagnostic criteria 2. Subacute VE • Meningo-encephalitis in the past • Dementia • Dysarthria • Pyramidal signs • Extrapyramidal signs • Slowly progredient illness (typically, 1 to 6 years duration) • CSF: increased cell count and protein • Neuropathology: Inflammatory foci (parenchymal and perivascular collections of lymphocytes), Reactive astrocytosis, Neuronal loss

  6. Diagnostic criteria 3. Chronic VE • Dementia • Dysarthria • Pyramidal signs • Extrapyramidal signs • No progression within the past 3-5 years • No other likely diagnosis • Neuropathology: Microcysts replacing micronecrotic foci, Brain atrophy, Gliosis, Neuronal loss, Residual inflammation in the parenchyma and meninges

  7. Science

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