[Prion diseases].
Prion is an ubiquitous membrane protein in mammals, which is mainly synthesized in central nervous system. Prion diseases are the result of an accumulation of prions having acquired a resistance to the physiological degradation and an infectious capacity. Human prion diseases are very rare diseases including sporadic Creutzfeldt-Jakob disease (the most frequent form manifesting as a presenile dementia), familial transmissible spongiform encephalopathies and two juvenile transmissible forms: iatrogenic Creutzfeldt-Jakob secondary to treatment with human extractive growth hormone and variant Creutzfeldt-Jakob disease resulting from bovine spongiform encephalopathy food transmission. Knowledge of the underlying prion biology has led to preventive measures which offer today a reasonable guarantee against the juvenile forms.
Billette de Villemeur
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Service de neuropédiatrie, hôpital Trousseau, 26, avenue Docteur-A.-Netter, 75012 Paris, France. thierry.billette@trs.ap-hop-paris.fr
D003937:Diagnosis, Differential; D006801:Humans; D015994:Incidence; D017096:Prion Diseases / Q000188:drug therapy / Q000473:pathology / Q000635:transmission; D011379:Prognosis