TERMIUM Plus®

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FAMILIAL CREUTZFELDT-JAKOB DISEASE [1 record]

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Alphabetical list of terms

Record 1 2012-05-03

English

Subject field(s)
  • Epidemiology
  • Genetics
DEF

Inherited form of Creutzfeldt-Jakob disease which shows an autosomal dominant pattern of disease segregation associated with a gene mutation.

OBS

If onset is usually at a slightly younger age (± 50), it generally shows clinical and pathological features analogous to the sporadic form.

French

Domaine(s)
  • Épidémiologie
  • Génétique
DEF

Forme héréditaire à transmission autosomique dominante de la maladie de Creutzfeldt-Jakob associée à une mutation du gène de la prion-protéine.

OBS

Elle survient plus précocement que la forme sporadique (âge moyen : 50 ans). D'évolution plus longue, elle présente des caractéristiques cliniques et neuropathologiques analogues à la forme sporadique.

Spanish

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