EXPERIMENTAL TRANSMISSION OF CREUTZFELDT-JAKOB-DISEASE AND RELATED DISEASES TO RODENTS

Sporadic Creutzfeldt-Jakob disease (CJD) with 129M/M, and iatrogenic a nd familial CJD with E200K and M232R, showed similar clinicopathologic features, a synaptic type deposition of PrPCJD, and high transmission frequencies to mice. Sporadic patients with 129M/V or 129V/V, and mut ation cases with V180I, showed slightly different features and low or null transmission frequencies to mice. Hereditary cases with P102L, P1 05L, A117V, Y145stop, and insertions had different features but all de monstrated a long clinical duration and the presence of PrP plaques. T he experimental transmission to mice of these mutant forms was difficu lt, except for one-third of the cases with P102L. CJD and related dise ases, even those that are hereditary, may thus be divided into two dif ferent groups, those that are easily transmissible and those that are either difficult to transmit or nontransmissible.