|
|
|
|
We present a synthesis of clinical,neuropathological,and biological details of the National Institutes of Health series of 300 experimentally transmitted cases of spongiform encephalopathy from among more than 1,000 cases of various neurological disorders inoculated into nonhuman primates during the past 30 years.The series comprises 278 subjects with Creutzfeldt-Jakob disease,of whom 234 had sporadic,36 familial,and 8 iatrogenic disease;18 patients with kuru;and 4 patients with Gerstmann- Straussler-Scheinker syndrome.Experimental transmission rates were highest for iatrogenic Creutzfeldt-Jakob disease(100%),kuru(95%),and sporadic Creutzfeldt-Jakob disease(90%),and considerably lower for most familial forms of disease(69%).Incubation periods as well as the durations and character of illness showed great variability,even in animals receiving the same inoculum,mirroring the spectrum of clinical profiles seen in human disease.Infectivity reached average levels of nearly 10 median lethal doses/gm of brain tissue,but was only irregularly present(and at much lower levels)in tissues outside the brain,and except for cerebrospinal fluid,was never detected in bodily secretions or excretions. |
|