Creutzfeldt-Jakob disease history and symptoms

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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:

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

  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)
  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)
  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)
  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.