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Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents
Abstract:
An unusual case of spongiform encephalopathy was transmitted directly from a human to rats and mice. After serial passages, incubation periods were shortened to about six months in rats and four months in mice. Clinical symptoms were similar in rats and mice, including ruffled fur, arched back, bradykinesia, and hind limb paralysis. Pathologically, a spongy state, proliferation of astrocytes, and neuronal changes were observed. Electron microscopic observation of the parietal cortex of rats and mice disclosed many membrane-bound vacuoles in the neuropil, predominantly in dendrites. In the pons of mice, intramyelinic vacuoles and accumulation of extracellular fluid were prominent. The clinicopathological symptoms of the affected animals resembled those in other experimental spongiform encephalopathies, especially scrapie.
Insights
This study details the direct transmission of human spongiform encephalopathy to rodents. Affected rats and mice showed similar neurological symptoms and pathological brain changes, resembling experimental scrapie.
Area of Science:
- Neuroscience
- Pathology
- Infectious Diseases
Background:
- Spongiform encephalopathies are fatal neurodegenerative diseases.
- Human transmission of these diseases to animals is rare and poorly understood.
- Previous research has established animal models for studying these conditions.
Purpose of the Study:
- To investigate the direct transmission of a human spongiform encephalopathy to rodent models.
- To characterize the incubation period, clinical signs, and pathological changes in experimentally infected animals.
- To compare the induced disease with known experimental spongiform encephalopathies.
Main Methods:
- Direct inoculation of affected human brain material into rats and mice.
- Serial passages of the infectious agent within rodent hosts.
- Clinical observation for neurological signs.
- Pathological examination including light and electron microscopy.
Main Results:
- Successful transmission of spongiform encephalopathy from human to rats and mice.
- Shortened incubation periods after serial passages (approx. 6 months in rats, 4 months in mice).
- Consistent clinical signs (ruffled fur, bradykinesia, paralysis) and neuropathology (spongiosis, astrogliosis, vacuolation) observed in rodents.
- Ultrastructural analysis revealed prominent neuropil and intramyelinic vacuoles.
Conclusions:
- Human spongiform encephalopathy can be experimentally transmitted to rats and mice.
- The induced disease in rodents shares key clinicopathological features with other experimental spongiform encephalopathies, particularly scrapie.
- This model provides a valuable tool for studying human spongiform encephalopathy pathogenesis and potential therapeutics.