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Unclassified: Spongiform Encephalopathies

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Laboratory Diagnosis of Infectious Diseases Principles and Practice

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Abstract

Disease: Scrapie of sheep and goats, transmissible mink encephalopathy, chronic wasting disease of captive mule deer and elk, and the human diseases kuru, Creutzfeldt-Jakob, and Gerstmann-Straussler syndrome.

Etiologic Agent: Nature unknown.

Source: Unknown.

Clinical Manifestations: Insidious onset with neurologic abnormalities progressing to dementia, listlessness, emaciation, and death.

Pathology: Limited to one organ, the brain; all cases exhibit status spongiosus, neuronal loss, and astrogliosis. The occurrence of amyloid plaques is observed in kuru and Gerstmann-Straussler syndrome and a few cases of Creutzfeldt-Jakob disease.

Laboratory Diagnosis: Primarily based on clinical signs, postmortem neuropathology, transmission studies, and presence of SAF and PrP in brain extracts.

Epidemiology: Scrapie and Creutzfeldt-Jakob disease occur worldwide. The other diseases are focal and all can occur in a familial manner.

Treatment: None known.

Prevention and Control: None known.

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Merz, P.A., Wisniewski, H.M. (1988). Unclassified: Spongiform Encephalopathies. In: Laboratory Diagnosis of Infectious Diseases Principles and Practice. Springer, New York, NY. https://doi.org/10.1007/978-1-4612-3900-0_42

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