CJD was first described in 1920 by Hans Creutzfeldt, a German neurologist and neuropathologist . The condition was described as a “peculiar focal illness of the central nervous system” discovered in a 23-year-old woman.
In 1921, Alfons Jakob, a German neurologist, published two articles on the anatomical findings of the disease. In 1923, he published another article describing a few cases that turned out to be fatal due to rapidly progressive dementia . At the time, Dr. Jakob felt that his patient’s symptoms were similar to Dr. Creutzfeldt’s reported case. As a result, this condition was later named Creutzfeldt-Jakob disease (CJD) in honor of the two doctors by Clearance J. Gibbs, an American microbiologist . Even though Dr. Creutzfeldt eventually did not agree that his cases matched those of Dr. Jakob’s, his name continues to be associated with the disease [2, 12]
In 1982, Stanley Prusiner, an American neurologist and biochemist, isolated prions and found that they were also present in healthy people and animals. Prusiner learned that prions were folded differently from regular proteins but could ‘infect’ the normal proteins, causing neurodegenerative diseases in humans.
For this landmark discovery, Prusiner was awarded the Nobel Prize in Physiology or Medicine in 1997, but he faced intense opposition. At the time, many in the medical community refused to accept the concept of infectious proteins causing degenerative diseases of the central nervous system. However, in time, Dr. Prusiner was proved right, and research on prions continued. Not one to rest on his laurels, he and his team established the role of prions in the spread of mad cow disease in 2004 [2,7].
Research on prions led to more understanding of CJD and many other neurodegenerative disorders. Although there has been progress, CJD is 100% fatal, and there is much left to uncover about prion diseases.