La persistente sombra de la enfermedad de las vacas locas

Los optimistas están proclamando que está desapareciendo la enfermedad de Creutzfeldt-Jakob (ECJ) variante, forma humana y siempre fatal de la encefalopatía espongiforme bovina (EEB) o "enfermedad de las vacas locas". Obviamente, dado el nivel de sufrimiento y ansiedad pública que ha causado la ECJ variante, la posibilidad de que esté retrocediendo es una noticia bienvenida, pero ¿es verdad?

La ECJ pertenece a la familia de lo que llamamos enfermedades priónicas, un grupo único de enfermedades neurodegenerativas que pueden transmitirse. Aunque la naturaleza precisa de la transmisión de la enfermedad sigue siendo incierta, un suceso clave en estos desórdenes es la conversión de las células normales de las proteínas priónicas en una forma anormal que parece ser el principal (aunque no el único) componente de la infección.

La ECJ variante se describió por primera vez en 1996, tras intensas actividades de vigilancia llevadas a cabo por la Unidad Nacional de Vigilancia de la ECJ (NCJDSU) del Reino Unido en Edimburgo. Esta nueva forma de enfermedad priónica tenía características clínicas y patológicas peculiares, y ocurrió en pacientes jóvenes que pertenecían a un solo subgrupo genético.

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