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Creutzfeldt-Jakob disease - Symptom

Alternative Names

Transmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt disease

Symptoms:

Additional symptoms that may be associated with this disease:

Once symptoms appear, the disorder progresses rapidly and may be confused with other types of dementia -- like Alzheimer's disease. Both forms of CJD, however, are distinguished by extremely rapid progression from onset of symptoms to disability and death.

Signs and tests:

A neurological and motor system examination shows muscle twitching and spasm. There is a strong startle response. Muscle tone may be increased, or there may be weakness and muscle wasting (loss of muscle tissue). There may be abnormal reflexes or an increase in the response of normal reflexes.

There is loss of coordination related to visual-spatial perception changes and changes in the cerebellum, the area of the brain that controls coordination (cerebellar ataxia). An eye examination shows areas of blindness that the person may not realize are present.

Tests used to diagnose this condition may include:

Ultimately, the disease can only be confirmed by brain biopsy or autopsy.

  • Reviewed last on: 8/6/2007
  • Daniel Kantor, M.D., Director of the Comprehensive MS Center, Neuroscience Institute, University of Florida Health Science Center, Jacksonville, FL. Review provided by VeriMed Healthcare Network.

References

Llewelyn CA, Hewitt PE, et al. Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 2004;363:417-421.

Peden AH, Head MW, et al. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004;264:527-529.

Brown P, Will RG, Bradley R, Asher DM, Detwiler L. Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease: Background, Evolution, and Current Concerns. EID. January-February 2001; 7(1):6-16.

Noble J. Textbook of Primary Care Medicine. 3rd ed. St. Louis, Mo: Mosby; 2001:1551-1569.