Creutzfeldt-Jakob disease natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Once symptoms develop, Creutzfeldt-Jakob disease is rapidly progressive and almost always fatal. Early symptoms are often non-specific. Patients' relatives usually report rapidly progressive dementia, personality changes, uncontrolled sporadic laughter, and sleep disorders. The majority of patients (85% of patients) die within 1 year of symptom-onset. Common complications of Creutzfeldt-Jakob disease include overwhelming infections, congestive heart failure, or respiratory failure.

Natural History

  • Once symptoms develop, Creutzfeldt-Jakob disease is rapidly progressive and almost always fatal.
  • Early symptoms are often non-specific. Patients' relatives usually report rapidly progressive dementia, personality changes, uncontrolled sporadic laughter, and sleep disorders.
  • The majority of patients (85% of patients) die within 1 year of symptom-onset, often due to complications succh as overwhelming infections, congestive heart failure, or respiratory failure.[1]

The following table demonstrates distinguishing features for classic and variant Creutzfeldt-Jakob disease:

Characteristic Classic CJD Variant CJD
Median age at death 68 years 28 years
Median duration of symptoms 4 to 5 months 13 to 14 months
Common clinical manifestations Dementia, early neurologic signs Psychiatric symptoms, painful dyesthesiasis, delayed neurological signs
Periodic sharp waves on EEG Present Absent
"Pulvinar sign" on MRI Not reported Usually present
"Florid plaques" on neuropathology Rare / absent Abundant
Immunohistochemical analysis of brain tissue Variable accumulation Marked accumulation of protease-resistance prion protein
Agent in lymphoid tissue Not detected Detected
Glycoform ratioo on immunoblot analysis of protease-resistance prion protein Not reported Marked accumulation of protease-resistance prion protein

Adapted from Belay E. Schonberger L. Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Clin Lab Med. 2002;22:849-62.[2]

Complications

Complications of Creutzfeldt-Jakob disease include the following:

Prognosis

  • The prognosis of Creutzfeldt-Jakob disease is very poor.
  • Patients usually die within 6 to 12 months of symptom-onset.
  • A few reports described individuals surviving beyond than 1 or 2 years after diagnosis.

References

  1. "http://www.cdc.gov/ncidod/dvrd/cjd/index.htm". Retrieved 17 February 2014. External link in |title= (help)
  2. Belay ED, Schonberger LB (2002). "Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy". Clin Lab Med. 22 (4): 849–62, v–vi. PMID 12489284.


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