On microscopic examination the distinctive finding is spongiform encephalopathy in gray matter throughout the brain and spinal cord. Severe neuronal loss and gliosis are also present and mild demyelination may occur. Ultrastructural changes include formation of intracytoplasmic vacuoles, which account for the spongy appearance. The disease is a seriously disabling and devastating disorder that progressively damages and destroys a person's memory, vision, and ability to think and move voluntarily, and function independently. Creutzfeldt-Jakob disease is one of a variety of transmissible spongiform encephalopathies (TSEs).
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Creutzfeldt-Jakob disease is not curable and is eventually fatal. CJD occurs worldwide at a rate of about 1 or 2 cases per million population per year. Most cases are sporadic, but 10 to 12% are inherited. The peak incidence is between 55 and 65 years of age, and the disease is rare before age 30. The average survival time is less than one year after onset of symptoms.
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