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Creutzfeldt-Jakob disease history and symptoms
Introduction
Overview
Overview
Common symptoms of Creutzfeldt-Jakob disease include uncontrolled sporadic laughter, progressive dementia, depression, personality changes, sleep disorders, and jerky movements (characteristic myoclonus).[1]
History and Symptoms
History and Symptoms
Common symptoms of Creutzfeldt-Jakob disease include the following:
- Uncontrolled sporadic laughter
- Jerky movements (myoclonus) (characteristic feature)
- Apathy
- Headache
- Depression
- Dementia
- Judgement difficulties[2]
- Memory loss
- Personality changes
- Hallucinations
- Seizures
- Insomnia or hypersomnia[3]
- Arthralgia
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.[4]
References
References
- β Haywood, AM. (1997). “Transmissible spongiform encephalopathies”. N Engl J Med. 337 (25): 1821β8. doi:10.1056/NEJM199712183372508. PMIDΒ 9400041. Unknown parameter
|month=ignored (help) - β Rabinovici, GD.; Wang, PN.; Levin, J.; Cook, L.; Pravdin, M.; Davis, J.; DeArmond, SJ.; Barbaro, NM.; Martindale, J. (2006). “First symptom in sporadic Creutzfeldt-Jakob disease”. Neurology. 66 (2): 286β7. doi:10.1212/01.wnl.0000196440.00297.67. PMIDΒ 16434680. Unknown parameter
|month=ignored (help) - β Landolt, HP.; Glatzel, M.; BlΓ€ttler, T.; Achermann, P.; Roth, C.; Mathis, J.; Weis, J.; Tobler, I.; Aguzzi, A. (2006). “Sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease”. Neurology. 66 (9): 1418β24. doi:10.1212/01.wnl.0000210445.16135.56. PMIDΒ 16682677. Unknown parameter
|month=ignored (help) - β Belay ED, Schonberger LB (2002). “Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy”. Clin Lab Med. 22 (4): 849β62, vβvi. PMIDΒ 12489284.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
