[Hereditary demyelination in mutant quaking mice (breeding and light microscopy)]. / Nasledstvennaia dismielinizatsiia u mutantnykh myshei kvoking (razvedenie i svetovaia mikroskopiia).

Data on breeding of mutant Quaking mice (MQM) and the results of light microscopy and morphometric examination of the central and peripheral nervous systems in them and in control mice varying in ages from 12 days to 4 months are presented. MQM were shown to have a decreased total volume of the white matter due to underdevelopment of myelin because of disturbed function of myelin-forming cells (oligodendrocytes). At the same time oligodendrocytes retain their capacity for proliferation and are normally located interfascicularly in the white matter having the same density of occurrence and the same average volume of the nucleus as in controls, but morphologically they are similar to oligodendroblasts. Another morphological feature of MQM consists of intensive vacuolation of their gray and white matter. However, light microscopy could not determine whether the vacuoles 1 to 9 microns in diameter were located intra- or extracellularly. No pathological changes in neurocytes, astrocytes or capillaries were observed.

[Hereditary demyelination in mutant quaking mice (electron microscopy study)]. / Nasledstvennaia dismielinizatsiia u mutantnykh myshei kvoking (élektronno-mikroskopicheskoe issledovanie).

Light and electron microscopic examinations of the nervous system in autosomal recessive mutant Quaking mice varying in ages from 12 days to 4 months revealed significant dysmyelination in which one of the most important morphological manifestations consisted of formation of "watery" astrocytes. The primary edema of astrocytes extended to other cerebral structures and produced their vacuolation, as a result of which significant disorders in the normal process of myelogenesis were observed, such as a decrease in the total volume of the white matter because of underdevelopment of myelin, disorders of the function of myelin-forming cells, oligodendrocytes. Pathological changes were also observed in some neurons and synapses. In contrast to the current opinion, the authors believe dysmyelination to be due not to primary involvement of oligodendrocytes but to edema of astrocytes. This does not rule out the genetic nature of this disease.

[Pathogenesis and pathomorphology of human leukodystrophies]. / Patogenez i patomorfologiia leikodistrofii cheloveka.

A review of foreign literature on the pathogenesis and pathomorphology of hereditary diseases of the human nervous system with the myelin involvement is presented. Five forms of leukodystrophies are mainly dealt with: (1) metachromatic, with the defect of their deposition in the form of a metachromatic substance; (2) globoid, with deficiency of galactoceramide beta-galactase enzyme catabolizing cerebrozides and with accumulation of the latter, particularly in the forming "globoid" cells; (3) sudanophilic, with sudanophilic degeneration of the myelin and obscure defect of the enzyme; (4) Pelizaeus-Merzbacher disease with insularly intact myelin; and (5) adrenoleukodystrophy with sudanophilic degeneration of the myelin and involvement of the adrenals. All the forms of leukodystrophies by the time of the onset of the disease are divided into prenatal, late infantile, juvenile, and adult.

[Reactive changes and the phagocytic activity of the glial cells in pathological processes in the central nervous system]. / Reaktivnye izmeneniia i fagotsitarnaia aktivnost' glial'nykh kletok pri patologicheskikh protsessakh v tsentral'noi nervnoi sisteme.

This review deals with the current status of reactive changes and phagocytic activity in various types of glia in the CNS. Data from literature and partly our own findings on the reactive processes in oligodendrocytes, astrocytes, microgliocytes, and "multipotential glia" as well as their relationship with mesenchymal cells, particularly with those of hematogenous origin are given. Glial reactions both in pathological processes (Walter degeneration, trauma, etc) and in different functional states during the development of the central nervous system are considered.