Introduction: subacute spongiform virus encephalopathies from the perspective of a neuroscientist

Abstract

Scrapie, a naturally occuring neurodegenerative disease of sheep and sometimes goats [25, 86, 907, 1050], is a prototypic disease for the whole group of the subacute spongiform virus encephalopathies. Kuru (Figs. 1–4) was the first human disease of this type to be discovered in 1957 by Gajdusek and Zigas (Fig. 1) [374, 381–382, 389–390, 508–509], and its discovery opened the whole field in the human biomedical sciences by the very realization of the fact that viruses may induce disease months or even decades after infections, and that these slow virus diseases are more compatible with classical degenerations of the nervous system than with inflammatory disorders of the brain. Incidentally, this viewpoint was a common knowledge in the veterinarian sciences perhaps from the time of transmission experiments of Cuille and Chelle [239–241] and seminal work by Sigurdsson [964].

“The most important works […] admit that we did not learn everything: but the amount of space devoted to the achievements, with occasional footnote mentions of what remained unknown — those very proportions suggested that we had mastered the Labyrinth, with the exception of few corridors — dead ends, no doubt, probably burried with rubble — whereas in fact we did not get as far as the entrance. Doomed forever to conjecture, having chipped a few flecks from the lock that sealed the gate, we delighted in the glitter that gilded our fingertips. But of what was locked we know nothing. And yet, surely, one of the first duties of a scientist is to determine the extent not of the acquired knowledge, for that knowledge will explain itself, but, rather, of the ignorance, which is the invisible Atlas beneath that knowledge.” Stanislaw Lem “His Master’s Voice”, translated from the Polish by M. Kandel, A Helen and Kurt Wolff Book, Harcourt Brace Jovanovich, Publ, San Diego — New York — London, 1983