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
- ↑ "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.