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Golgi and electronmicroscopic studies of spongifom encephalopathy

Department of Neurology (Drs. Landis and Williams), Massachusetts General Hospital, Boston, MA, and the Laboratory of Central Nervous System Studies (Dr. Masters), National Institutes of Health, Bethesda, MD.

Golgi impregnations of cerebral biopsies from two patients suffering from Creutzfeldt-Jakob disease (subacute spongiform encephalopathy) revealed striking loss of dendritic spines of pyramidal neurons and unusual focal spherical distensions of dendritic and axonal processes. Light and electronmicroscopic studies disclosed spongiform changes, which seemed to be caused by intracytoplasmic vacuoles and expansion of the plasmalemmal membranes of neurons and astrocytes. Although the diagnostic biopsies were performed at a markedly symptomatic stage of the disease, there was no evidence of neuronal cell loss. Neuronal changes in Golgi impregnations of cerebral cortex from hamsters infected with scrapie were essentially identical to those in the human biopsies. The loss of dendritic spines of pyramidal cells and spherical swellings of axons and dendrites thus seem to be characteristic of spongiform encephalopathies, and probably account in part for the clinical neurologic manifestations, which may be severe in the relative absence of neuronal death.

Address correspondence and reprint requests to Dr. Landis, Department of Neurology, Massachusetts General Hospital, Boston, MA 02114.

This study was supported in part by USPHS Grants Nos. NS 12005 and NS 15573. Dr. Landis is a recipient of an NINCDS Teacher-Investigator Award No. NS 00353.

Accepted for publication July 30, 1980.

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