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

Section of Neuropathology, Yale University School of Medicine, New Haven, CT, and the Division of Pathologic Anatomy, The Children's Hospital of Philadelphia, PA.

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 ultrastrutural 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.

Address correspondence and reprint requests to Dr. Manuelidis, Section of Neuropathology, Yale University School of Medicine, 333 Cedar Street, P.O. Box 3333, New Haven, CT 06510.

Supported by grants NS 12674 and AG 03106 from the National Institutes of Health, and by the Commonwealth Fund.

Received October 11,1988. Accepted for publication in final form January 26, 1989.

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