Prions, Clinical Features
Creutzfeldt-Jakob disease is one of the transmissible spongiform encephalopathies, a family of diseases affecting humans and animals (see Table 1). They are transmissible, in that susceptible animals inoculated with diseased tissue will develop a similar disease; spongiform, in that, under a microscope, small spaces (vacuoles) in brain tissue are invisible, giving the appearance of a sponge; and encephalopathies, in that they affect the brain.
The disease was first reported by Hans Creutzfeldt in 1920 and Alfons Jakob in 1921. A related, exclusively familial disease, Gerstmann-Sträussler-Scheinker syndrome, was reported in 1928. Creutzfeldt-Jakob disease is a dementia characterized by a rapid progression and a multitude of varying cognitive and motor deficits.
The first advance in understanding the diseases occurred with the recognition that kuru, a disease afflicting only the Fore people of New Guinea, caused similar changes in brain tissue. Kuru is a rapidly progressive dementia (over months) characterized by cerebellar degeneration, causing clumsiness and difficulty walking (ataxia), tremor, and slurred speech. It has been linked to the Fore's practice of eating the brains of deceased relatives. Dr. D. C. Gadjusek, of the National Institutes of Health, hypothesized that ingestion of brain tissue caused the disease, and demonstrated in 1966 that inoculation of brain tissue from kuru patients into chimpanzees' brains caused the disease. Shortly afterward transmissibility was also demonstrated for Creutzfeldt-Jakob disease and Gerstmann-Str;äussler-Scheinker syndrome. Gadjusek and a colleague, Baruch S. Blumberg, were awarded the 1976 Nobel Prize in Medicine for this work.
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