[Hereditary demyelination in mutant quaking mice (electron microscopy study)]

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.