The nature of a pathological process in the central nervous system in Vilyui encephalomyelitis defines it as a specific entity of demyelinative encephalomyelitis with a drastic prevalence of an alternative component. The bulk of the nerve parenchyma dies from circulatory incompetence due to blood vascular diseases, which are typical of the disease, as a peculiar angiopathy and progressive reduction in the microcirculatory bed. Homeostatic disorders in the myelinic membranes of central nervous fibers were found to be of value. The maintenance of homeostasis is implicated by adequate oligodendroglial function and copper metabolism. The inhabitants of the Republic of Sakha (Yakutia) are demonstrated to have oligodendroglial hypoplasia and exogenous and endogenous copper deficiency. This is suggested by low copper amounts in the natural environmental objects and in the hair of patients with chronic Vilyui encephalomyelitis.
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Rev Med Virol
October 2008
Department of Neurology, University of Illinois at Chicago School of Medicine, Chicago, IL 60612-7344, USA.
For more than a century, a type of human encephalomyelitis has been known to affect indigenous people in the Sakha Republic in the Vilyui River Valley in Russia. The clinical features, laboratory findings, neuropathology, epidemiology and search for a causative pathogen are reviewed. One of the agents (Vilyuisk human encephalitis virus; VHEV) implicated in Vilyuisk encephalitis, belongs to a separate clade of Theiler's murine encephalomyelitis virus (TMEV).
View Article and Find Full Text PDFVestn Ross Akad Med Nauk
April 1994
The nature of a pathological process in the central nervous system in Vilyui encephalomyelitis defines it as a specific entity of demyelinative encephalomyelitis with a drastic prevalence of an alternative component. The bulk of the nerve parenchyma dies from circulatory incompetence due to blood vascular diseases, which are typical of the disease, as a peculiar angiopathy and progressive reduction in the microcirculatory bed. Homeostatic disorders in the myelinic membranes of central nervous fibers were found to be of value.
View Article and Find Full Text PDFBiopsies from the cortex of the right frontal lobe of 3 patients with chronic Vilyui encephalomyelitis were examined light- and electron microscopically. The characteristic degenerative changes in the gangliocytes and especially in the nerve fibers are typical for this disease; particularly severe lesions were found in the myelin sheaths and to a lesser extent in the axons. Myelin sheaths lamella, external mitochondrial membranes and other organelles showed osmiophilic degeneration with their destruction and release of peculiar crystal-like formations having an elective localization in the processes cytoplasm and not in the perikaryon.
View Article and Find Full Text PDFCNS histopathology of 2 cases of Vilyui encephalomyelitis (with neurological symptomatology of 7-month and 3-year duration) is presented. The picture demonstrates severe focal degenerative changes of neurons combined with nonpurulent inflammatory reaction in the pia mater and around the blood vessels of the cerebral and spinal cord white and gray matter. Characteristic features of the disease include swelling of the neurons and their processes, asymmetric thickening of the nuclear membrane, ectopy of the nucleoli and their exit from the nucleus and from the cell, karyocytolysis; formation of spongious foci and fine gliofibrous scars; considerable atrophic, edematous, and dystrophic changes in conduction tracts of the hemispheres, brain stem and spinal cord.
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