Creutzfeldt-Jakob disease natural history, complications and prognosis: Difference between revisions
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*Early symptoms are often non-specific. Patients' relatives usually report rapidly progressive dementia, personality changes, uncontrolled sporadic laughter, and sleep disorders. | *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.<ref name="www.cdc.gov">{{Cite web | last = | first = | title = http://www.cdc.gov/ncidod/dvrd/cjd/index.htm | url = http://www.cdc.gov/ncidod/dvrd/cjd/index.htm | publisher = | date = | accessdate = 17 February 2014 }}</ref> | *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.<ref name="www.cdc.gov">{{Cite web | last = | first = | title = http://www.cdc.gov/ncidod/dvrd/cjd/index.htm | url = http://www.cdc.gov/ncidod/dvrd/cjd/index.htm | publisher = | date = | accessdate = 17 February 2014 }}</ref> | ||
The following table demonstrates distinguishing features for classic and variant Creutzfeldt-Jakob disease: | |||
{| {{table}} | |||
| align="center" style="background:#f0f0f0;"|'''Characteristic''' | |||
| align="center" style="background:#f0f0f0;"|'''Classic CJD''' | |||
| align="center" style="background:#f0f0f0;"|'''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 ono 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 | |||
|} | |||
<SMALL>Adapted from Belay E. Schonberger L. Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Clin Lab Med. 2002;22:849-62.<ref name="pmid12489284">{{cite journal| author=Belay ED, Schonberger LB| title=Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. | journal=Clin Lab Med | year= 2002 | volume= 22 | issue= 4 | pages= 849-62, v-vi | pmid=12489284 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12489284 }} </ref></SMALL> | |||
==Complications== | ==Complications== |
Revision as of 21:22, 22 March 2016
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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 ono 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.