Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD), rare fatal degenerative disease of the central nervous system. CJD occurs throughout the world at an incidence of one in every one million people. Among certain populations, such as Libyan Jews, rates are somewhat higher.

The disease was first described in the 1920s by the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob. CJD is similar to other neurodegenerative diseases such as kuru, a human disorder, and scrapie, which occurs in sheep and goats. All three diseases are types of transmissible spongiform encephalopathies, so called because of the characteristic spongelike pattern of neuronal destruction that leaves brain tissue filled with holes.