Facts About Creutzfeldt-Jakob Disease: CDC-OC


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Article title: Facts About Creutzfeldt-Jakob Disease: CDC-OC
Conditions: Creutzfeldt-Jakob Disease
Source: CDC-OC

Facts About Creutzfeldt-Jakob Disease

November 14, 1997
Media Relations Division
(404) 639-3286

  • Creutzfeldt-Jakob Disease (CJD) is a progressive neurological disorder which belongs to a group of degenerative neurologic diseases known as subacute spongiform encephalopathies.
  • Clinical features of CJD include a neurological presentation, with dementia, and a progressive cerebellar syndrome including ataxia, gait, and speech abnormalities. In most patients, these symptoms are followed by involuntary movements and the appearance of a typical diagnostic electroencephalogram tracing.
  • From 10 to 15 percent of CJD cases are inherited, but cases have been associated with the use of contaminated corneal transplants, electrode implants, dura mater grafts, and receipt of human growth hormone.
  • CJD occurs worldwide at a rate of about 1 case per million population per year.
  • The disease is found most frequently in patients 55-65 years of age, but cases can occur in persons older than 90 years and younger than 55 years of age.
  • In more than 85 percent of cases, the duration of CJD is less than 1 year (median: 4 months) after onset of symptoms.
  • CDC monitors the occurrence of CJD in the United States through periodic reviews of national mortality data.
  • On the basis of mortality surveillance from 1979 to 1994, the annual incidence of CJD remained stable at approximately 1 case per million persons in the United States.
  • In the United States, CJD deaths among persons younger than 30 years of age are extremely rare (fewer than 5 deaths per billion per year).

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