1920 CREUTZFELD, Hans Gerhard (1885 – 1964)
Ueber eine eigenartige herdförmige Erkrankung des Zentralnervensystems.
Creutzfeld-Jakob disease, spatic pseudoschlerosis, independently discovered by Jakob. (see No. 4722). English translation in No. 5019.14, pp. 97-112. Creutzfeld described a single case and later reported that "his cas…
GMN 4719.1 · https://historyofmedicine.com/id/1205
1921 JAKOB, Afons Maria (1884 – 1931)
Ueber eigenartige Erkrankungen der Zentralnervensystems mit bemerkenswertem anatomischem Befunde. (Spastische Pseudosklerose — Encephalomyclopathie mit disseminirrten Degenerationsherden.)
“Creutzfeld-Jakob disease”, spastic pseudosclerosis. Traditionally considered to have been independently discovered by Creutzfeld, but in the 21st century recognized as a discovery by Jakob alone. See also No. 4719.1.
GMN 4722 · https://historyofmedicine.com/id/1219
1923 JAKOB, Afons Maria (1884 – 1931)
Die Extrapyramidalen Erkrankungen: Mit Besonderer Berücksichtigung der pathologischen Anatomie und Histologie und der Pathophysiologie der Bewegungsstörungen.
Pages 218-245 represent Jakob's full clinical-pathologic description of the fifth patient that he first described in this work.The symptoms of this patient and the histopathopathologic illustrations correspond fully w…
GMN 12594 · https://historyofmedicine.com/id/14834
1957 GAJDUSEK, Daniel Carleton (1923 – 2008); ZIGAS, Vincent (1920 – 1983)
Degenerative disease of the central nervous system in New Guinea. The endemic occurrence of “Kuru” in the native population.
First description of Kuru, a disease occurring in natives of New Guinea. Cause of the disease was unknown. "It is now widely accepted that kuru was transmitted among members of the Fore tribe of Papua New Guinea via f…
GMN 4729.1 · https://historyofmedicine.com/id/1253
1959 HADLOW, W. J.
Scrapie and Kuru.
In 1959 Hadlow, a veterinarian, visited a medical exposition in England where Carlton Gajdusek posted pathological slides of autopsied Kuru brains and a clinical description of the illness. He realized that Gajdusek's…
GMN 10840 · https://historyofmedicine.com/id/13036
1965 GAJDUSEK, Daniel Carleton (1923 – 2008); GIBBS, Clarence Joseph Jr. (1924 – 2001); ALPERS, Michael
Attempts to demonstrate a transmissible agent in Kuru, Amyotrophic Lateral Sclerosis, and other subacute and chronic progressive nervous system degenerations in man. Addendum p. 46 in: Slow, latent, and temperate virus infections. NINDB Monograph No. 2. Edited by D. Carleton Gajdusek, Clarence J. Gibbs, Jr., and Michael Alpers.
In their Addendum on p. 46 the authors stated that 20 and 21 months post innocculation in the brain with brain material from Kuru patients two chimpanzees showed symptoms of an illness suggestive of Kuru. Digital facs…
GMN 13914 · https://historyofmedicine.com/id/16208
1967 GRIFFITH, John Stanley (1928 – 1972)
Self replication and scrapie.
Griffith discussed complex physical chemistry mechanisms by which the scrapie agent could arise from information encoded in protein structure which could then be transferred to other protein molecules. He also conside…
GMN 12833 · https://historyofmedicine.com/id/15079
1967 ALPER, Tikvah (1909 – 1995); CRAMP, W. A.; HAIG, D. A.; ET AL
Does the agent of scrapie replicate without nucleic acids?
This paper, which predated Griffith's' paper (No. 12833), demonstrated that the scrapie agent replicates without nucleic acids. Alper and colleagues irradiated scrapie infected mouse brain extracts with lethal ultravi…
GMN 12834 · https://historyofmedicine.com/id/15080
1968 MATTHEWS, John D.; GLASSE, Robert M. (1930 – 1993); LINDENBAUM, Shirley
Kuru and cannibalism.
Medical anthropologists Lindenbaum and Glass demonstrated that Kuru was transmitted in New Guinea by cannibalism--particularly by eating the brains of infected victims, which were the reservoir of prions. Order of aut…
GMN 10841 · https://historyofmedicine.com/id/13037
1971 GAJDUSEK, Daniel Carleton (1923 – 2008); GIBBS, Clarence Joseph Jr. (1924 – 2001)
Transmission of two subacute spongiform encephalopathies of man (Kuru and Creutzfeldt–Jakob disease) to New World monkeys.
Following Hadlow's suggestion (1959), Gadjusek was able to transmit Kuru and Creutzfeldt–Jakob disease to primates through cerebral inoculations. Similarity in the clinical course of the diseases and in the cellular p…
GMN 4729.2 · https://historyofmedicine.com/id/1255
1982 PRUSINER, Stanley Benjamin (1942 – )
Novel proteinaceous infectious particles cause Scrapie.
In 1997 Prusiner was awarded the Nobel Prize in Physiology or Medicine "for his discovery of Prions - a new biological principle of infection." In his 1982 paper Prusiner proposed a completely novel explanation for th…
GMN 10140 · https://historyofmedicine.com/id/12329
1982 PRUSINER, Stanley Benjamin (1942 – ); BOLTON, David C.; MCKINLEY, Michael P.
Identification of a protein that purifies with the Scrapie prion.
Research with the biochemist Bolton enabled Prusiner to discover and characterize the specific protein causing prion disease. This paper was dated December 24, 1982. Nearly simultaneously, Prusiner and the same co-aut…
GMN 10842 · https://historyofmedicine.com/id/13038
1982 GAJDUSEK, Daniel Carleton (1923 – 2008); MASTERS, Colin Louis (1947 – )
The spectrum of Creutzfeldt-Jakob disease and the virus-induced subacute spongiform encephalopathies. IN: Smith & Cavanagh, editors, Recent advances in neuropathology, Vol. 2., Chapter 6, pp. 129-163.
The authors reported a detailed and complete analysis of the original slides of the brain done by Afons Jakob, and provided a partial translation into English, with a detailed analysis of Jakob's clinical description,…
GMN 12595 · https://historyofmedicine.com/id/14835
1986 LUGARESI, Elio (1926 – 2015); MEDORI, Rosella; GAMBETTI, Pierluigi; ET AL
Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei.
The authors coined the name Fatal Familial Insomnia to describe a family cohort of individuals who were dying from a prion illness causing an inability to sleep. This disease has been characterized as one of the most …
GMN 10843 · https://historyofmedicine.com/id/13039
1996 WILL, R. G.; IRONSIDE, J. W.; ZEIDLER, M.; ET AL
A new variant of Creutzfeldt-Jakob disease in the UK.
During the 1990s England was plagued with cases of Bovine Spongiform Encephalopathy (BSE) seen in cows, popularly known as "Mad Cow Disease." Then physicians in England started noticing an uptick in cases of what look…
GMN 10844 · https://historyofmedicine.com/id/13040
1996 PRUSINER, Stanley Benjamin (1942 – ); TELLING, Glenn C.; PARCHI, Piero (1951 – ); DE ARMOND, Stephen J.; ET AL
Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity.
The authors showed that the " 'normal prion protein' in the brains of living mice can be converted into different forms depending on the type of abnormal human prion that initiated the conversion. The result is differ…
GMN 12835 · https://historyofmedicine.com/id/15081
2004 PRUSINER, Stanley Benjamin (1942 – ); LEGNAME, Giuseppe; BASKAKOV, Ilia V.; NGUYEN, Hoang-Oanh B.; ET AL
Synthetic mammalian prions.
The authors modified Koch's Postulates within the context of prion disease. To do so the followed these steps: 1) They created recombinant mouse prion proteins in an E. coli and polymerized them. 2) They proved that t…
GMN 12836 · https://historyofmedicine.com/id/15082
2013 MEAD, S.; GANDHI, Sonia; BECK, Jon; COLLINGE, John; ET AL
A novel prion disease associated with diarrhea and autonomic neuropathy.
Order of authorship in the original paper: Mead, Gandhi, Beck, Collinge. Collinge was the main author. Digital facsimile from nejm.org at this link. (Thanks to Juan Weiss for this entry and its interpretation.)
GMN 11045 · https://historyofmedicine.com/id/13241
2014 PRUSINER, Stanley Benjamin (1942 – )
Madness and memory: The discovery of prions- a new biological principle of disease.
Prusiner discovered prions, the agent causing scrapie in sheep and goats, mad cow disease, and Creutzfeldt-Jakob disease in humans.
GMN 7624 · https://historyofmedicine.com/id/9796
