Creutzfeldt-Jakob disease natural history, complications and prognosis

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

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

Template:WikiDoc Sources

[www.cdc.gov-1] “http://www.cdc.gov/ncidod/dvrd/cjd/index.htm”. Retrieved 17 February 2014. External link in |title= (help)

[pmid12489284-2] 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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