Creutzfeldt-Jakob disease history and symptoms

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

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

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

↑ Haywood, AM. (1997). “Transmissible spongiform encephalopathies”. N Engl J Med. 337 (25): 1821–8. doi:10.1056/NEJM199712183372508. PMID 9400041. Unknown parameter |month= ignored (help)
↑ 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): 286–7. doi:10.1212/01.wnl.0000196440.00297.67. PMID 16434680. Unknown parameter |month= ignored (help)
↑ Landolt, HP.; Glatzel, M.; Blättler, 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): 1418–24. doi:10.1212/01.wnl.0000210445.16135.56. PMID 16682677. Unknown parameter |month= ignored (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.

[Haywood-1997-1] Haywood, AM. (1997). “Transmissible spongiform encephalopathies”. N Engl J Med. 337 (25): 1821–8. doi:10.1056/NEJM199712183372508. PMID 9400041. Unknown parameter |month= ignored (help)

[Rabinovici-2006-2] 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): 286–7. doi:10.1212/01.wnl.0000196440.00297.67. PMID 16434680. Unknown parameter |month= ignored (help)

[Landolt-2006-3] Landolt, HP.; Glatzel, M.; Blättler, 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): 1418–24. doi:10.1212/01.wnl.0000210445.16135.56. PMID 16682677. Unknown parameter |month= ignored (help)

[pmid12489284-4] 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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Creutzfeldt-Jakob disease history and symptoms

Overview

History and Symptoms

References

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