L’Ombre planante de la maladie de la vache folle

Les optimistes déclarent que la variante de la maladie de Creutzfeldt-Jakob (MCJ), sa forme humaine, toujours fatale, l’encéphalopathie bovine spongiforme (ESB), aussi connue sous le nom de « maladie de la vache folle », est en train de décliner. Étant donné le degré de souffrance et d’angoisse causé dans le public par la MCJ, il est évident que la possibilité que cette maladie soit sur le déclin est une nouvelle bien accueillie. Mais est-ce bien le cas ?

La MCJ appartient à la famille de ce que l’on appelle les maladies des prions, un groupe unique de maladies dégénératives qui peuvent être transmises. Bien que la nature exacte de la transmission de la maladie reste incertaine, un des événements clé dans ces désordres est la conversion des cellules saines de la protéine du prion en une forme anormale qui semble être le principal (si ce n’est le seul) composant de cette infection.

La variante de la MCJ fut décrite pour la première fois en 1996, suite à une surveillance intensive entreprise par l’Unité nationale de surveillance de la MCJ (NCJDSU) du Royaume-Uni, située à Édimbourg. Cette nouvelle forme de la maladie du prion présentait des caractéristiques cliniques et pathologiques particulières et se présentait chez de jeunes patients appartenant à un sous-groupe génétique unique.

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