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A Creutzfeldt-Jakob disease agent (Echigo-1 strain) recovered from brain tissue showing the 'panencephalopathic type'
S Mori1, C Hamada, T Kumanishi
1Department of Neurology, Niigata University, Japan.
Abstract:
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.
Insights
Researchers identified a transmissible pathogen causing panencephalopathic Creutzfeldt-Jakob disease (CJD) in guinea pigs and hamsters. This study details the pathogen
Area of Science:
- Neurology
- Pathology
- Virology
Background:
- Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder.
- The panencephalopathic subtype of CJD is prevalent in Japan, unlike in North America or Europe.
- Histologic evidence of gray and white matter degeneration is key for diagnosing panencephalopathic CJD.
Observation:
- A transmissible pathogen, designated Echigo-1 strain, was isolated from a patient with panencephalopathic CJD.
- Serial passage in Hartley guinea pigs led to degenerative brain changes, primarily in the thalamus.
- A substrain with a shortened latent period emerged by the fourth passage.
Findings:
- The Echigo-1 substrain induced severe degeneration in the thalamus and cerebral cortex of Golden Syrian hamsters.
- Experimental transmission demonstrated the pathogen's ability to cause CJD-like pathology in animal models.
- Comparative analysis with other experimental CJD models was performed.
Implications:
- This research contributes to understanding the pathogenesis of panencephalopathic CJD.
- The findings may aid in developing diagnostic or therapeutic strategies for CJD.
- Establishes a new animal model for studying this specific CJD subtype.