Subacute spongiform encephalopathy: Electron microscopic studies

Abstract

SUMMARY Electron microscopic changes in brain biopsies from two cases of subacute spongiform encephalopathy are described. Large vacuoles seen in astrocytic and neuronal processes were surrounded by one or more concentric membranes. Dense-core vesicles were found in axonal endings and in nerve cell perikarya; these particles could represent catecholamine-containing vesicles.

Spongiform Encephalopathy of Long Duration: A Family Study

“…While technical artifact could not be absolutely excluded, the finding within such vacuolated neurites of curled fragments of membrane, sometimes in multi-vesicular configuration ( Fig. 4 d e), strongly supported our impression that this patient had a genuine spongiform encephalopathy of the type described by several authors (Bignami and Forno, 1970;Bubis et al, 1972;Gajdusek, 1972;Kidd, 1967).…”

Value of brain biopsy in neurodegenerative disease in childhood.

“…It is unfortunate that no ultrastructural clues exist in the present series, since without electron microscope studies the presence of various forms of inclusion bodies and virus-like particles (Bubis et al, 1972), as well as mitochondrial abnormalities (Suzuki and Rapin, 1969;Sandbank and Lerman, 1972), would be missed. We would certainly advocate ultrastructural examination if not culture studies in future biopsies.…”

“…Although in skilled hands brain biopsy is generally a safe procedure, we think that the only practical justification for what is essentially a damaging and potentially harmful procedure (Groves and M0ller, 1966;Bubis, Goldhammer, and Braham, 1972) is if it assists with prognosis and genetic counselling. That such a small proportion of biopsies yields diagnostically valuable information for these purposes is somewhat disappointing.…”