[Pathomorphology of the nervous system in beta-thalassemia major]

Pathomorphology of symmetrical areas of various departments of the central nervous system was studied in 8 children aged 3--8 years who had died of beta-thalassaemia major. In 5 patients transient cerebral vascular disorders occurred against the background of hemolytic crisis, in 2 -- phenomena of polyradiculoneuritis, in 1 -- spinal desorders with lower flaccid paraparesis. Vessles were found to be damaged by the pathological process first. Impairment of permeability of vascular membranes, hemorrhages and edema of the brain substance were observed. As to neurons lesions, along with normal neurons there were nerve cells with reactive and dystrophic changes. In pulpous nerve fibres periaxonal alterations and Wallerian degeneration predominated. The combination of the erythroblastic process with secondary impairment of the metabolism, toxicosis, hypoxaemia contributed to the mixed character of the morphological changes, which accounted for the polymorphism of neurological symptoms.