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Creutzfeldt-Jakob Disease

more about Creutzfeldt-Jakob Disease


  • Creutzfeldt-Jakob disease (CJD) is a fatal disorder of the central nervous system characterized by rapidly progressive dementia. This is a form of human spongiform encephalopathy. Other forms include kuru, fatal familial Insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Spongiform encephalopathy may occur in certain animals such as cows, sheep and so on. Mad cow disease, also referred to as Bovine spongiform encephalopathy (BSE), is an example of spongiform encephalopathy which can affect cows.

  • Creutzfeldt-Jakob disease occurs in people over the age of 50. The peak incidence is in between the years of 60 and 65. Dementia is present in all cases.
    1. Impaired memory
    2. Intellectural dysfunction
    3. Personality change
    4. Myoclonus
    5. Impaired muscle balance and coordination
    6. Gait disturbance
    7. Aphasia
    8. Psychiatric symptoms -- anxiety, Depression and hallucination
    9. Visual disturbance
  • There are new variants of Creutzfeldt-Jakob disease, which are characterized by early onset (mean age is 30 years old), no family history of disease, early psychiatric symptoms and prolonged course of the disease. Some laboratory tests reveal that the etiologic agents of the new variant Creutzfeldt-Jakob disease and mad cow disease are similar, but it is not proven whether the disease is transmitted from cattle to humans.

  • The etiologic agent is a protein particle produced in human cells called prion. Over 85% of the cases are in sporadic form, which occurs as a result of the mutation of the gene coding for the prion. About 5 to 10 % of the cases are genetic. Creutzfeldt-Jakob disease can arise from the direct contact with the infected tissues (such as corneal transplantation) or certain medical procedure, but this is very rare.

  • The symptoms of Creutzfeldt-Jakob disease are similar to those with other neurological diseases such as Alzheimer's disease, Parkinson's Disease and encephalitis. There is no single laboratory test to confirm the existence of Creutzfeldt-Jakob disease. Only a brain biopsy or autopsy can confirm the diagnosis. A post mortem brain exam reveals a sponge like appearance due to degradation of brain tissue.

  • There is no curable treatment for Creutzfeldt-Jakob disease. The disease progresses rapidly, causing most patients die within 1 year.

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