CJD research at the IBB
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease that belongs to the category of the transmissible spongiform encephalopathies. CJD is transmissible within a species but also in between species for example from humans to apes. Our laboratory has extensive experience with the classic neuropathological diagnosis of CJD. New probes presently allow the development of antibody based laboratory tests. In Belgium there are no clinical laboratories where this type of laboratory test is routinely performed. The Health Council has recommended the development of a methodology to enable a differential diagnose of CJD.
We developed immunohistochemical protocols to detect at least one of these markers in brain with a very high degree of reliability.
The materials necessary to develop these tests are available in the brain-bank and CSF sample collection of our laboratory or by collaboration with other laboratories. These collections contain samples of CJD patients as well as samples of patients that suffer from other neurodegenerative diseases, for example Alzheimer's disease and healthy controls. Our laboratory also possesses antibodies against the CJD markers and the technical know-how about immunoassays and immunohistochemical techniques.
Technical directions and forms
Obtained results of the 14-3-3 detection
Obtained sensitivity and specificity
Overview tested samples
Diagnostic criteria for sporadic CJD (Rotterdam, 1998)
Contact
Links
Obtained results of the 14-3-3 detection
Obtained sensitivity and specificity: detection of Biochemical markers
Using all detected 14-3-3 bands
Sensitivity: 98%
Specificity
to other dementias: 95%
to all categories: 91%
If weak positive is regarded as negative
Sensitivity: 89%
Specificity
to other dementias: 99%
to all categories: 95%
Overview tested samples
| 14-3-3 Analysis | |||
|---|---|---|---|
| Positive | Negative | Total | |
| CJD definite | 94 | 2 | 96 |
| CJD probable | 42 | 1 | 43 |
| CJD possible | 12 | 15 | 27 |
| CJD familial | 4 | 2 | 6 |
| TOTAL CJD | 140 | 5 | 145 |
| Alzheimer’s disease | 6 | 120 | 126 |
| Dementia (not AD) | 8 | 141 | 149 |
| TOTAL Dementia | 14 | 261 | 275 |
| Other neurological disorders | 13 | 126 | 139 |
| No final diagnosis | 10 | 101 | 111 |
| Paraneoplastic disorders | 4 | 15 | 19 |
| Psychicatric disorders | 1 | 16 | 17 |
| Vascular disorders | 1 | 12 | 13 |
| Viral encefalitis | 14 | 14 | 28 |
| TOTAL Other disorders | 43 | 284 | 327 |
| TOTAL | 209 | 565 | 774 |
| Percentile results | |||
| Positive | Negative | Total | |
| CJD definite | 98% | 2% | 100% |
| CJD probable | 98% | 2% | 100% |
| CJD possible | 44% | 56% | 100% |
| CJD familial | 67% | 33% | 100% |
| TOTAL CJD | 97% | 3% | 100% |
| Alzheimer’s disease | 5% | 95% | 100% |
| Dementia (not AD) | 5% | 95% | 100% |
| TOTAL Dementia | 5% | 95% | 100% |
| Other neurological disorders | 9% | 91% | 100% |
| No final diagnosis | 9% | 91% | 100% |
| Paraneoplastic disorders | 21% | 79% | 100% |
| Psychicatric disorders | 6% | 94% | 100% |
| Vascular disorders | 8% | 92% | 100% |
| Viral encefalitis | 50% | 50% | 100% |
| TOTAL Other disorders | 13% | 87% | 100% |
| TOTAL | 27% | 73% | 100% |
Diagnostic criteria for sporadic CJD (Rotterdam, 1998)
1. Diagnostic symptoms
I - rapidly progressive dementia
II - Clinical symptoms
A - Myoclonus
B - Visual or cerebellar problems
C - Pyramidal or extrapyramidal features
D - Akinetic mutism
III - Investigations
A - Typical EEG
B - positive 14-3-3
2. Diagnostic status
Probable CJD: I and 2 of II and III A and/or B
Possible CJD: I and 2 of II and duration less than 2 years
Definite CJD: neuropathologically/immunohistochemically confirmed
Contact
| Prof. Dr. Patrick Cras | Ing. Bart De Vil |
|---|---|
| mail prof. Cras | mail Bart De Vil |
| Tel: +32 3 821 57 57 | Tel: +32 3 265 25 97 or +32 3 265 26 05 Fax: +32 3 265 26 69 |
| Laboratory of Neurobiology University of Antwerp CDE Universiteitsplein 1, Building T, Room 5.20 BE-2610 Antwerp, Belgium |
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