Transmission of Alpers' disease (chronic progressive encephalopathy) produces experimental Creutzfeldt-Jakob disease in hamsters.

We successfully and serially transmitted to outbred and inbred strains of hamsters the brain tissue of a 2 1/2-year-old girl with a chronic progressive encephalopathy (Alpers' disease) characterized postmortem as a spongiform encephalopathy. In all hamster strains we produced a spongiform encephalopathy. The light and ultrastructural changes in the brain of hamsters, as well as the clinical signs of experimental disease, are identical to those obtained in transmission experiments of human Creutzfeldt-Jakob disease (CJD). CJD infection may be more widespread than previously recognized and can be manifested in infancy.