Altered Cell Membranes in Creutzfeldt-Jakob Disease

Abstract

Quantitative microchemical assays with histologic controls were used to study biochemical structural components in specimens of frontal cortex from patients with Creutzfeldt-Jakob disease (two cases) as compared with normal cortex (eight cases). Neuronal damage at an early stage of the disease was evidenced by depletion of ribosomal organelles (RNA per cell) and disintegration of axodendritic membranes and synapses (loss of ganglioside sialic acid). Distention of neuronal processes in association with cytoplasmic membrane-bound vacuoles resulted in status spongiosus (decreased total solids per unit volume). While astrocytes hypertrophy and proliferate, neurons are progressively destroyed leading to secondary myelin destruction (cerebroside depletion). Chloroform: methanol soluble proteins (proteolipid proteins) were increased, possibly representing membrane fragments that electron microscopists have shown to occupy intracellular vacuoles in neurons and postulated to harbor the infectious agent.