PrP Synaptic buttons synaptic deposits spongiosis kuru plaques

CJD onderzoek in het Instituut Born-Bunge

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.

Technische richtlijnen en formulieren

Resultaten van de 14-3-3 bepaling

Sensitiviteit en specificiteit
Overzicht stalen

Diagnostische criteria voor sporadische CJD (Rotterdam, 1998)

Contactgegevens

Links

 

Resultaten van de 14-3-3 bepaling

Sensitiviteit en specificiteit: detectie Biochemische merkers

Gebruik makend van alle 14-3-3 banden

Sensitiviteit: 98%

Specificiteit

t.o.v. andere dementies: 95%

t.o.v. alle categoriën: 91%

Indien zwak positief als negatief wordt beschouwd:

Sensitiviteit: 89%

Specificiteit

t.o.v. andere dementies: 99%

t.o.v. alle categoriën: 95%

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Overzicht stalen

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%

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Diagnostische criteria voor sporadische CJD (Rotterdam, 1998)

1. Diagnostische symptomen

I - snel progressieve dementie

II - klinische symptomen

A - myoclonus

B - visuele of cerebellaire problemen

C - pyramidale of extrapyramidale tekens

D - akinetisch mutisme

III - onderzoeken

A - typisch EEG

B - positieve 14-3-3

2. Diagnostische status

Probable CJD: I en 2 van II en III A en/of B

Possible CJD: I en 2 van II en dit gedurende een periode van minder dan 2 jaar

Definite CJD: neuropathologisch/immunohistochemisch bevestigd

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Contactgegevens

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
Laboratorium voor Neurobiologie
Universiteit of Antwerpen CDE
Universiteitsplein 1, Gebouw T, Lokaal 5.20
BE-2610 Antwerpen, België

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Links

The Belgian surveillance network

NeuroCJD

EuroCJD

UK CJD surveillance

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