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NEUROLOGY 1989;39:1337
© 1989 American Academy of Neurology

A Creutzfeldt-Jakob disease agent (Echigo-1 strain) recovered from brain tissue showing the 'panencephalopathic type' disease

S. Mori, MD, C. Hamada, MD, T. Kumanishi, MD, N. Fukuhara, MD, Y. Ichihashi, PhD, F. Ikuta, MD, T. Miyatake, MD and T. Tsubaki, MD

Department of Neurology, Brain Research Institute, Niigata University, Niigata City, Japan (Drs. Mori, Fukuhara, Miyatake, and Tsubaki)
Neuropathology, Brain Research Institute, Niigata University, Niigata City, Japan (Dr. Kumanishi)
Experimental Neuropathology, Brain Research Institute, Niigata University, Niigata City, Japan (Dr. Ikuta)
Department of Virology, Niigata University School of Medicine, Niigata City, Japan. (Drs. Hamada and Ichihashi)

We used histologic evidence of degenerative changes in both the gray and white matter of the brain to diagnose a patient as having the panencephalopathic type of Creutzfeldt-Jakob disease (CJD). This type of CJD is relatively common in Japan, but not in North America or Europe. We recovered a transmissible pathogen (Echigo-1 strain) from an autopsy specimen of the patient's brain and passed it serially in Hartley guinea pigs. After a long latent period, it caused degenerative changes, mainly in the thalamic area of the guinea pig brain. On the 4th passage, a substrain emerged with a short latent period. When cross-transmitted to Golden Syrian hamsters, this substrain induced severe degeneration in both the thalamus and cerebral cortex. We compare our results with those for other experimental CJDs produced by other types of this disease.

Address correspondence and reprint requests to Dr. Mori, Department of Neurology, Brain Research Institute, Niigata University, Asahimachi 1, Niigata City, Japan 951.

Supported in part by a grant from the Ministry of Health and Welfare, Japan.

Received December 20, 1988. Accepted for publication in final form April 25, 1989.




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